Thursday, December 30, 2010

Small World

I can't even describe how nice it was to be away from here but I learned alot about my life while I was gone.  I learned just how small my world is.   I don't know if I can explain this well enough for anyone else to understand but here goes. 
     I am reading a book right now called Room by Emma Donoghue.  It's about a girl who has been abducted and hidden away in a shed for 7 years.  9 months After an incident with her abductor, she has a baby who now lives with her hidden away in this shed that has no windows and a locked door.  It measures 11' square but he provides her with a flushing toilet,fridge,tv, table, bed and wardrobe.  He drops off food every now and again but only the essentials.  The story is how she tries to raise this boy creating a world that seems normal for him so he isn't scared and doesn't feel like he is being held captive.  The boy has no idea that any world exists beyond their tiny world in the room. 
     Sometimes, I feel like my world is like this, small and foreign.  Sometimes, I feel like I too am trying to live in a world that is not normal but I pretend it is.  If I don't know what I'm missing then it's really okay, I find my groove and there I stay.  I think my kids are totally normal until I see that all the pain and fatigue they experience is not what everyone else outside our world experiences.  Pain, nausea, fatigue are not things anyone else has to deal with everyday but it's our normal.  Activities that you don't even think about like shopping, working, eating, playing can become a real problem for my kids. 
    When I escape this world, like I did this past week, I think of how much I have isolated myself from the world.  Maybe it's all the sickness and disease we have no choice but to deal with that has made us transparent, even invisible at times.  I guess, it really does feel like we are being held captive when we see what a 'normal' life is like.   We become too big for the world, people get tired of hearing about all the problems that never go away and so I retreat to where it's safe in our little 11' x 11' room that we have become so familiar and comfortable with. 
     Tonight, the kids had to go to their dad's for the start of the New Year's holiday and here I am sitting home, the house is dead quiet, and it's a little lonely.  I think about all the things I haven't accomplished the past few years and talent I've wasted perhaps.  I think about the life I don't have when my kids are gone.  They are my life.   I worry that I need to do something to increase the size of my world but what...what would that be?
     So, I guess while I have some quiet time, I will try to figure some things out, contemplate a future that feels like it already has a ceiling on what I can or can't do.  There has to be more.  I had this talk with Shelbie, she feels the impact of her small world as well.  It's just one side effect of chronic illness that I hadn't anticipated or fully understood until now.  It's a little hard to think it through and I fear I am not doing as good a job as I did when the kids were younger and could hide their disease from them.  I never wanted SDS to define their life but in so many ways, it was sneaky and has taken so much from them already.

Sunday, December 26, 2010

Week One Post IVIG

Well, it's been just over a week since the last infusion.  Shelbie is still pretty tired and sleeps alot which is good.  We haven't done too much but play games and eat.  Last night, she was sick with stomach flu kinds of stuff but I loaded her up with Zofran and sent her to bed.  We were able to avoid anything too nasty and she was feeling better this morning. 
      Tomorrow will be our biggest day yet.  We will be going to the West Edmonton Mall again but this time to shop.  The kids received some gift cards and money so they are really excited.  Fashion here in Canada is a little different than Idaho and they have quite a few stores here that we don't have so that will be fun to break into a different style.  I will have to rent a wheelchair or scooter for Shelbie, I can't imagine there is anyway she will be able to get around otherwise. 
    I'm already getting depressed at the thought of coming back.  There are so many reasons why we need to get out of where we are at but so many reasons to stay.  I have come to realize on this trip just how small our world is.  I'm trying to decide if I like that or not.  At any rate, whether we want to come home or not, we will be leaving in two days...it just hasn't been long enough.

Thursday, December 23, 2010

Much needed break

Coming to Edmonton has been just what the kids needed, especially Shelbie.  It has done wonders for her spirits.  She is really tired but hanging in there and enjoying the break from doctor appointments and worries.  She was able to have fun in the wave pool today but had to rest a lot because she is still really weak and runs out of steam.  She took at nap in a lounge chair by the pool then spent some time relaxing in one of the three hot tubs. 
     I will admit, going for bloodwork everyday is comforting.  It is nice to know where she stands so we can be proactive instead of feeling like we are blindsided all the time.  That's the only hard thing about being here.  We can't just run to the clinic or hospital to check on things.  I can always assume that the IVIG continues to work it's magic, dump the immune system but bring up the platelets. I know the first thing we will want to do when we get home is go for a blood check. 
     On the flight here, I had to be sure to take plenty of precautions for her poor immune system.  I packed anti bacterial wipes and we used them to wipe down the armrests, trays and anything else we came in contact with in hopes that no one, especially Shelbie would get sick.  It was disgusting to say the least the amount of crud that came off those armrests and trays with just one swipe!  If I didn't have OCD before, I have it now!  It started me thinking about all the things we touch on a daily basis that appear to be clean but lurking under that disguise of cleanliness is a mess of germs.  Ewwww!  So for now, everything is good...too good.  I don't ever want to come home.  I want to pretend that the last few months have just been a bad dream!

Tuesday, December 21, 2010

Update

Platelets are coming up today.  She is at 80 which is okay but still really low.  Good enough for us to go home for Christmas though.  We will have to take some precautions because her white count is super low and her hemoglobin is suffering too.  This means that she can hardly walk 20 ft without feeling winded and extreme fatigue.  We will have plenty of time to make our way through the airport and I have packed lots of antibacterial wipes, lotions and anything else that will keep her germ free.
      I feel so relieved that we can go.  It has been a busy afternoon trying to get ready.  I have people coming to stay the week at our house so the house can't just be clean but squeaky clean!  The kids are really looking forward to this trip.  Lately, the tension and fatigue has been choking us.  It feels like we are getting a pass out of jail. 
     I will continue to update and post from Canada.  Hopefully Shelbie won't have any problems while we are there.   It would be nice if we were going with better functioning bone marrow but that's life!  I hope I will be able to leave our worries here.  As soon as we get back we will be bombarded with hospitals again.  Shelbie will more than likely have surgery soon after we return to place a port or pic line, at least that is the talk now.  Spencer will undergo some very uncomfortable allergy testing, worse than last time and the following week, we head to Boise for a visit to our Gastroenterologist and St. Lukes Hospital.  Somewhere amongst all of that, there will be another infusion for Shelbie and unless we try something new, another bout with aseptic meningitis.  So, I really hope we get a lot of rest and recouperation out of the next 6 days.

Monday, December 20, 2010

A fun surprise!

Sunday, Shelbie had some visitors.  I have known them for a year or two but not really known them well. We mostly just say 'hi' when we see each other.  They have a daughter just younger than Shelbie who has had some rough health problems too.  We have gotten to know each other much better over the last few weeks.  She has come by on a couple of occasisions to visit and called to share well wishes for Shelbie. 
     She is a school teacher of a 4th grade class and had them color a big mural for Shelbie.  Sunday, she brought it over along with some tasty treats.  It was so fun to see the great artwork from such caring children who have never met Shelbie.  When they unrolled it, it stretched from our kitchen all the way to the back door.  It was such a sweet surprise and really cheered us up.  I appreciate her thoughtfulness a lot!


Big thanks to all those kids who shared their warm thoughts with us and to this great family who made it all happen!

Sunday, December 19, 2010

Second Verse...Same as the first

This time she brought some comforts from home.  Her happy little Sock Monkey slippers

Surrounded in her favorite feel good color- Zebra.  My good friend made the awesome pillowcase cover!  She rested much better with her own blanket and pillow and actually looks pretty good prior to the infusion.  Too bad she didn't continue to look so great!
Yup, another long weekend in the hospital.  Shelbie's infusion went well, Saturday morning went well.  Saturday night...not so well!  I had tried all day to keep the celebrex and loratab on board so that we could stay on top of any potential side effects but by 6:30 our luck had run out.  Still, I kept trying to avoid the hospital.  By 10:30 she was screaming at me in pain and then the nightmare began.  She had run out of patience and longsuffering and I was the only one to receive the fallout.  Even though I knew she was just exhausted and in so much pain, it was still hard to hear those all too familiar words, "You don't even care!"
       I do care, I care too much.  I cared so much that I kept trying to convince her and myself that this time would be different.  I spent too much time in denial and the pain got way out of control.  There goes that mother of the year award I was so hoping to achieve!  Oh well, there's always next year. 
      Anyways, we had the same nurse as last time in ER and she was very helpful.  One of our favorite family doctors was the ER doc, (there are 4 providers at the family clinic where we go and we love every one of them!) He is always really good with the kids and it felt nice to let someone else take over who actually knew what they were doing. 
      Today, Shelbie's platelets are up to 30 which is a nice improvement.  Staying on top of the pain here at home now and so far so good.  I am beat, in so many ways.  I haven't had but 4-5 hours of sleep since Friday night.  I am trying to get on top of laundry, packing the Christmas presents and clean the house before things get crazy around here.  We are still planning to leave this week to go home but I have arranged for some friends to stay at the house while we are gone so I need to get things shaped up for them.  Hopefully, Shelbie will steadily improve, the boys will stay healthy and I can shake this crazy virus that has settled in. 

Friday, December 17, 2010

Deep thoughts...

Well, didn't sleep hardly at all last night, tried but Shelbie has been so kind to share her rotten cold with me so that has been pleasant, then all the worrying about today, tomorrow, next week, next month, next year.....At some point, I need to get an emergency shut off switch in my head so I can just stop all the overtime spent in thinking. 

Last night, I was catching up on emails from the SDS group and other families I follow through their blogs.  Three SDS kids go to transplant the first part of January.  One sweet boy was suppose to start Dec. 20 but his donor was in a car accident so he will have to wait a little longer for his new marrow.  Hopefully, he will be able to stay strong until the donor has recovered enough to undergo the procedure. 

Another family I read about has a son with leukemia.  He has been battling it for a year now and has just under two years of chemo left to go.  I had all sorts of feelings and emotions come over me as I read about this family and their experience.  They go to the same Oncologist we do.  She was raving about how great the nurses are at the clinic and mentioned the nurse who I had my run in with earlier this week.  They are kind and patient and always doing fun things for this boy on chemo days.  To read her post and compare it to my post about the frustrations this week, you would have a hard time believing we were talking about the same person, same clinic...

At first I felt angry when I read it.  Why couldn't they just be nice to us?  Why is it that everytime I try to go through the right channels to talk to our doctor, we get the run around?  Then the most horrifying thought of all.  Maybe if my kids had cancer we would be treated better.   I know, it's an awful thought to even consider.  Of course I don't want my kids to have cancer but cancer is something everyone knows and hates!  The nurses at the clinic know cancer.  They know how it acts, they know how their patients feel, they know it is the most horrible thing in the world to have especially when it happens to a kid.  They know how the blood will react with each poisoning treatment of chemo.  They know cancer in and out.
        I just want someone to know my kids inside and out the way they know cancer.  Here in lies the problem and I guess it's my problem not the innocent nurse on the other end of the phone who was just trying to do her job regardless of how well I thought she did it.  It gets really lonely being the only one to advocate for my kids.  I guess I am a little tired of doing this all alone.  I am the one who has to stay on top of things, stay in step with the doctor so we don't miss anything. I feel like everyday is a fight and a worry.  Every sniffle, sneeze and strange ache has to be considered as a potential threat to the life of my kids.  No one else understands what this feels like, not even the best trained nurse who knows everything there is to know about cancer but we don't have cancer, we only have SDS.   I like to think that someday this will change and the voice on the other end of the phone will be compassionate and helpful and sometimes on rare occasions, it is.  But while I know SDS inside and out and hate it, no one else does so how can I really expect anything from anyone but to recognize that they are doing their best?
       In the meantime, here we sit in the hospital.  Shelbie's platelets are at 5 and the IVIG is streaming into her veins.  She is sleeping well, the premeds have knocked her out.  She has a low grade fever and her blood pressure is super low but other than that, no other side effects yet.  I am pretty tired, took a 10 min nap earlier this morning and that felt good.  So glad that Spencer is feeling well and is my big support at home.  I try not to give him too many responsibilities because I don't want to steal away his youth but I do appreciate him taking Sam to school, picking him up and running a few errands.  Sam has also tried to be patient with all this running around.  He is wired a little differently from the other two kids and anything that deviates from his regular schedule produces a lot of anxiety for him so I appreciate how hard this is on him too.  I have the sweetest kids, I wouldn't trade them for anything...not even cancer!

Thursday, December 16, 2010

Infusions tomorrow

Well, our doctor called me tonight, he was able to get the information he needed on the pending tests and since her counts have dropped so quickly, she will be in the hospital tomorrow for IVIG.

 I felt so much better after talking to him.  Just to clarify things, I really like him and I think we work well together as  team.  He is always willing to explain things 50 different ways until I understand what is happening and I appreciate that quality in him. He is also happy to listen to my thoughts, worries, anxieties etc and we work through them together.  Just wanted to be clear that I have never had a complaint about him.   He tries to stay up on SDS which I also appreciate.   I hope my last post didn't reflect anything poorly about him, I will say that he still has some staffing issues to consider though.  So, we spoke briefly about the next few days and we will not do surgery before Christmas, it will more than likely take place after the holidays.  Relieved about that as well. 

Tomorrow will be a long day.  We will try doing IV fluids before the infusion and run it slow again to see if we can avoid the nasty side effects.  It's hard to predict what will happen, Shelbie is sick tonight, her cold has gotten much worse just in a couple of hours so hopefully that will not put her at a disadvantage.  In a sense, it's a big relief to get this taken care of before the weekend but I am still not looking forward to it.  Shelbie of course is handling it okay or at least seems to be.  I guess that is to be expected.  The patient always seems to cope better than the mom looking on, wishing so much I could stop this all from happening.  I can see that she feels better mentally knowing something will happen to get her out of the danger zone even if that something is not very pleasant.  So here we go again...deja vu!

Tuesday, December 14, 2010

Frustrations

Today has not been without it's frustrations.  I often think about all the things I think would be cool to do.  One of those things I would love to do is gather all the doctors in the country together and let them know what it feels like to be the patient or the mother of the patient.  The first thing I would tell them is that their Secretary will make or break them as a doctor and their practice.  Secondly, their nurses are the key factor in my decision to stick around as a patient or find somewhere else to go regardless of how great the doctor really is.  Third, some things can't not be discussed with a nurse or secretary so get on the phone and talk to me!

I have had to deal with all three nuisances today and it really irks me.  I know I should be more patient but I am tired.  A doctor generally has no idea what it's like to live our life.  They don't know what it's like to be the mom, trying to keep a sick kid calm.  They don't know how stupid and unprepared I feel because I only understand one third of all this stuff and if they get all medical on me, then we get lost and anxious because not knowing or not understanding something leaves me feeling powerless. Yes, knowledge is power.  Power in the sense that we have hope, something to hang our hat on, keep our feet grounded instead of our minds spinning things to the worst case scenario. 

After finding out Shelbie's platelets are dropping low again, well below normal, I called to our doctor's office.  I asked the secretary if she could have the doctor call me, and here is the run around I get.

" Well, he is busy all day, he won't have time to call you but maybe tomorrow." 
Don't speak for the doctor, give him the message and let him decide if he can or should call me back...."This is very important regarding Shelbie and her low platelets again, we need to know how to move forward with this."
Big annoyed sigh, "Well, I guess I can pass this along to a nurse."

Arrgghhhhhhh..... sit and wait....the nurse calls back....
"I understand your daughter has low platelets again.  The doctor is waiting for a test to come back so he isn't going to do anything until the results are in next Monday."
"What do those test results have to do with the fact her platelets are low other than it decides if she gets a port or not?  Regardless, she still needs an infusion and we are leaving the country next week so the sooner the better.  I already discussed this with the doctor last week."
"Well, he thinks there is something more serious going on so you will have to wait."
"Serious like what?  This is the first I've heard this."
"I don't know, that's just what he said.  I have had a lengthy discussion with him."
"If you had a lengthy discussion then what is he talking about?"
"Well, you'll have to talk to him about that."
"Okay, so when can I talk to him?"
"You'll have to wait until next Monday when the tests are back and then he will do the IVIG."
"We leave the country on Wednesday by plane.  I need to know that her counts are stable and she is over the meningitis side effects before Wednesday and that won't happen if the infusion doesn't even take place until Monday afternoon.  In the meantime, what are we suppose to do in a day when her platelets are at 1 or 0?"
"Well Ma'm, there is no way her platelets are going to drop to 1 or 0, that just doesn't happen. And aseptic meningitis is such a rare side effect, she will be fine."
About to blow my friggin top but trying really hard not to let this nurse have 42 years of  pent up anxiety and anger "In our world, it happens all the time!  It happened three weeks ago...1!  A platelet count of 1! It happened every other week 3 years ago and 5 years before that! And, every time she gets IVIG she has the meningitis side effects, EVERY TIME."
"I'm sure it wasn't exactly 1 but she isn't having symptoms so she's fine."
"Have you seen her?  She has broken blood vessels all over her stomach, that's not fine... Ya know, I have the doctors cell phone number so tomorrow when she is at 1, I will call him on that number.  Thanks so much for your help." Honestly, I was using my sweetest voice ever then quickly ran to the garage so I could scream in my car. Even now as I type this, I want to gnash my teeth to powder.

Seriously, life is hard enough without some know it all nurse who thinks she can guard the doctor like he's some Hollywood star.  He's just a guy with a degree.  Our world famous doc in Seattle is far more accessible than any local doc...just saying. It's not like I was calling to complain about a sore throat!   If he thought there was something more serious going on than what I have been led to believe then that is just wrong for me to find out from some nurse who we have never met and has no clue what is going on.

 It's the little things like this that really make this unbearable!  I am going to need a moment of zen, realign my chakras and do a lot of meditation to recover from two lousy hours today that could have been prevented with a little better communication.  I hate letting myself get so annoyed but I have reached the end of that proverbial rope!   screaming myself to sleep now.....

...Not Ready!

Mentally not ready for another weekend of infusions, meningitis, blah, blah, blah.  Shelbie opted out of blood work yesterday as we normally do and went in this morning instead.  We didn't even guess the counts which has become our traditional fun while we wait.  I actually had no idea what to expect.  I think I had probably convinced myself that they would still be in the normal range and we would not have to do this again.  Platelets are at 64.  She has another cold so her white cells are elevated to 3...still very low but elevated for her! 

So, now what, you ask?  Well, we are awaiting one test that will measure the levels of her Immunogamma Globulin.  If this is low, then we can expect surgery to place a catheter in a major vein just up from her heart with a port under her skin so that she will have an easier time getting the medications and testing she needs.  This also means that treatment will be every two to three weeks for a very long time...a very long time!  Everyday we go to the hospital is a nightmare anymore, she is running out of decent veins from which to draw from.  She looks like an addict, bruises all over her arms and now with low platelets again, it's worse.  At any rate, these tests won't be back until Monday so the doctor is debating what to do in the meantime. 

We have made plans to go home for Christmas but we will not be able to go if Shelbie is not somewhat stable.  Having the infusion sooner would be ideal so we can get the side effects taken care of before trying to get through 2-3 airports next week.  For today, we have to be patient.  Be patient, oh what fun that is.  I am so tired and never prepared for the inevitable but as in all trials we come upon, we will grin and bear it or at least front our way through it.

Sunday, December 12, 2010

Are you ready?

This week, I heard someone ask Shelbie if she was ready for whatever was coming her way?  She nodded yes.  I can't seem to get that question out of my mind.  What would my answer be if the question had of been asked of me?  I am afraid I would have had to say "no".  I try to be prepared for 'the worst case' but when the worst case happens, I always feel blindsided.  I'm not sure you can ever be prepared for all the emotion, fatigue, frustration and confusion that comes with any trial though we try to do our best.  When we leave the walls of our home, we try to front our way through but that can often be quite a disconnect from the wrestling that takes place with a situation on any given day inside our home and hearts.  It's just not that easy to be ready.

I guess I find some solace in remembering how I have handled the worst things that have happened so far.  I always thought the worst thing I would ever have to face was being divorced; single and all alone.  It felt hopeless for me to make a living and support my kids.  Nearly 6 years later, I'm not the best provider financially but I have survived and aside from moments, I have done pretty good for myself, in fact I have probably surprised a few people! 

Losing my job was the next worst thing I couldn't imagine surviving.  Ask a few close people how I handled that news and they will tell you I did not handle that first week with much grace.  It seemed impossible to handle and overwhelming to know how I could manage financially.  It's been almost 18 months and I am still trudging along...albeit very tired, but still trudging. 

So, now to our current situation of poor health and other personal things I haven't mentioned, it's hard to imagine being ready for anything worse though I'm pretty sure there is more to come and overcome.  No one these days will escape the worst thing I'm afraid.  I have learned one thing, each trial we go through, prepares us for the next one.  I guess that's where the preparation lies.  Whatever comes next, I have to believe I have already received all I will need to get us through. 

We are anticipating a big week.  I should hear on the kids' mitochondrial testing.  The results have been back for nearly two weeks but the doctor has been at Hematology conferences and she said the results were very complex and wanted to spend more time before discussing them.  This is week three of the IVIG infusion for Shelbie.  The medication generally lasts 3 weeks so we could see a crash of the platelets this week.  Her big problem the last few days has definitely been her hemoglobin.  It is so low that she can hardly do a thing.  One 1/2 hour outing sends her to bed for hours.  Her white count is nothing to speak of either so it's been challenging.  The third thing we are looking at this week is her IgG levels (immunoglobulin)  If that level is low, then the option becomes to place a 'port' in Shelbie and do IVIG infusions indefinitely, maybe the rest of her life.  The port would allow a less painful way to do the infusion and take blood but requires a surgery to place the catheter.  Lots happening this week, should prove to be very interesting...as if we can stand anymore interest in our life!  ;-)

Thursday, December 9, 2010

Ear, Nose and Throat

Just because one kid is sick, doesn't mean the others take time off.  We spent the afternoon at the Ear, Nose Throat doc.  Spencer has such intense allergies and so many things he is allergic to that he will need to undergo more specific testing before they can start the weekly injections to try to control his allergic reactions.  He is less than thrilled about this so we will wait until right after Christmas. The testing will involve a series of injections and last 3-4 hours and leave him feeling pretty sick so it won't be too fun.  On top of that, it will take place the day after we get back from GI testing in Boise.  I planned it then because he will already be missing a few days of school so what's one more that week?

Sam also saw the doctor but there is nothing they can do for him.  He suffers much like a CF kid does with a thick, stringy mucus that his body has a hard time managing.  It's not as bad as a CFer but still a nuisance for him.  I wasn't sure that SDS kids could have the same kind of struggles with mucus as those suffering with CF so that will be something I will have to look into.

Shelbie really needs to have her tonsils out but in light of the recent problems with her, the doctor won't touch her with a 10' pole.  Here is the rock and hard place for Shelbie.  Oncology says that each time she gets a virus or bacterial infection from here on out, it will aggrevate her ITP and cause her platelets to fall so, it's like she is a walking time bomb.  The ENT believes that she is colonizing bacteria in her huge tonsils and perpetuating infections, sore throats and colds.  All these issues are creating a vicious circle of illness.  It' just something that we will have to get use to yet try not to get all paranoid about it. 

I did not escape the bad news today either.  I have had issues with hearing loss for some time now.  He diagnosed me today with Otosclerosis and possible Menieres disease.  Otosclerosis is a hardening and calcification of the bones in the inner ear.  It is progressive and leads to total deafness.  Surgery is an option but comes with risks and may leave you deaf anyways.  It is a genetic disease and is the leading cause of deafness in young adults.  Menieres disease doesn't have a cure either and causes dizziness, ringing in the ear and a fullness in the ear with pain.  I have all of these problems from time to time.  Our plan is to recheck my hearing in 6 months.  They will know by the hearing tests when it is time for surgery.  In the meantime, I get to buy myself a new ear....a hearing aid.  Merry Christmas to me!  That's just exactly what I've always wanted, a shiny black hearing aid with blue tooth!  They've come a long way.  I guess I better brush up on my sign language! ;-)

The more I get to know families who have children with SDS, the more I discover the problems the mothers have with their health.  I know a handful who have similar issues with arthritis and so on that I have.  I guess it makes sense, if I have passed on such horrific genes to my kids, then I must have some issues myself.  Genetics is so interesting.  I must have been the lucky one because my siblings are pretty healthy.  Oh well, better me than them.

Wednesday, December 8, 2010

On and on

A strange thing happens when we go through stuff like we have in the past few weeks.  I seem to manage just fine as we go through the worst of the crisis.  It must be adrenaline that kicks in and keeps me going because as soon as the initial worry passes, I drop off like a fly in November. 
     Today has been the hardest to get through yet; since Shelbie's counts dropped two weeks ago.  I feel like a balloon that has been deflated.  At 6pm I was ready to call it a night.  I don't often feel like this but it always happens after we get through something big.
     To update on Shelbie, her counts are still dropping in increments of 8000.  The IVIG has been in her system one week as of this Friday.  It is suppose to be effective for 2-3 weeks so it's too bad that we are already seeing the falling counts.  I have learned a lot about the auto immune process.  The biggest thing I have learned is that it is completely unpredictable.  No one can judge how long this is going to go on.  It could end as fast and as unexpected as it started.  It 's been really interesting to line up all her blood reports and chart out how the counts change with platelets, IVIG and nothing.  I think I am starting to see a pattern that might provide cues to what could happen in the future. 
      The hardest thing is waiting for the shoe to drop.  Of course we try to live a normal existence but it's hard always wondering what will happen next and when it will happen.  I have tried to get her to plan one thing to do each day so she has a purpose to get up in the morning but it's really important that she doesn't overexert herself and that's hard to find the balancing point.  We are still trying to get that right.
      

Monday, December 6, 2010

It was nice while it lasted

Well, just got back from the hospital.  The falling trend of the platelets has begun.  It was really nice enjoying high counts for a week.  As it looks right now, depending on how fast they fall, she will have another infusion sometime towards the end of the week or first part of next week.  We will see the doctor tomorrow and make a plan.  My plan is that when we do another infusion, I want her admitted so that it can run slower than last time and have lots of fluids on board as well.  Taking more time to do it might eliminate a lot of the side effects.  We will also discuss the option of a pic line or port so she doesn't have to get stuck so much.  Today was really tough for them to find a vein.  The chemo she had three years ago has changed the size of her veins and they are really small now.  Couple that with the fact she is covered in bruises and some scar tissue is beginning to form, blood draws are no longer that simple.   We will have a couple of busy days in the next few with lots of appt. planned; Ear Nose Throat, Family Doc and Oncology...

It's amazing to me just how fast ones mind can take a crummy situation and put it in a bubble so that you can move forward each day in something that resembles normal without really thinking about the pending doom.  That is pretty much how the last week has felt.  Lots of people at church asked about Shelbie and what the future holds.  I just rattle off some facts like they really don't matter.  I feel immune from being sad or overwhelmed which is good but creates a bit of a disconnect for other people who are clearly worried and distressed on our behalf.  Then we see results like today and the bubble bursts and we are reminded that this isn't going to just go away on its own.  A wise philosopher once said that we should be like a little kid who plays with bubbles, they don't cry when one bursts, they just set out to blow another one.  Today, we will just have to work on that new bubble to insulate us from the intensities that will surely come. 

Friday, December 3, 2010

Whaa hoooo!

So, Shelbie and I made our daily trip up to the hospital for bloodwork this morning.  Her platelets are at 158!  Awesome!!  We were so excited to see that number!  It was nice to feel the relief of a normal count.
Everyday, we have a little contest with the nurses to see who can come closest to guessing the platelet count. I have won twice this week.  I was only two away today and two away on Wednesday.  Do I know my kid or what?!
      On the downside though, her white count has dropped again to well below normal and her monocyte count is through the roof.  Neither one of those is a good thing but there is not much we can do.  In fixing one thing, we created a host of other problems.  As long as I can keep her virus and bacteria free, she should be okay.  It is promising to see such good counts one week after IVIG.  After speaking to the doc, he explained that the IVIG should stay in her system for 2-3 weeks.  After that time, it is likely that her counts will tank again and we will do another infusion, get sick for a week, have a week or two of feeling okay then do it all over again.  This is a chronic problem rather than an acute one which comes on fast and ends quickly. Of course this is just one scenario, the one we have been familiar all of her life.  It is always possible that God has something different in store for her like stable counts that last for months and no more IVIG for a long while.  It's nothing we can predict...

Thursday, December 2, 2010

I Just Realized...

It is strange when the very realities of life really couldn't be seen before but from nowhere wake you up like a call at 3:00am.  I just realized that Shelbie really is sick.  For the last couple of years, she has become more and more tired, run down and never seems to feel well.  For the last couple of years, I have tried to coax her into finding a life worth living.  I have done that again this week.  In my mind, I think if I can just get her back into a new routine of 'normal' then she will fair better or if she gets off the couch she lays on all day and does something, then she will feel better.  I made her go to work for an hour on Monday and Tuesday.  Tonight I convinced her that going to Idaho Falls with us would be a nice break from the house.  Spencer wanted to find a snow board jacket and I had some returns to make.  It's fun to hang out with my kids shopping.  She decided that was a good idea. 

We all headed in and not even 10 min. into our first store, she is done.  Her head hurts, she's dizzy, she feels sick to her stomach and like she just ran a marathon.  I got really frustrated because I guess deep down, I don't want to see her sick.  I push her so hard because somehow, I think if she is 'doing' something than everything is okay. I have never wanted my children to define themselves by the illnesses they keep.  They are bigger than that! 

By the third stop, she was crying.  "You just don't get it do you?  I am sick.  I feel sick all the time, I can't do the things you want me to do.  I can't walk around these stores.  I just can't but you don't seem to care."
I care but I don't want to accept that she really is sick and she may not get any better than this.  I like to pretend that things are going to get better but maybe it's time to face reality.  I need to stop making things better.  I can't make things better.  I guess that's what hurts the most.  I use to be able to kiss it all away.  I just want to see my kids without the accessory of suffering. 

Wednesday, December 1, 2010

Why Me?

This has been an interesting couple of weeks.  We have had a lot of problems compressed into a short amount of time.  There were moments when I was so exhausted I didn't think I would even make it one more minute but then there were other times, when I have felt more energy than ever before.  So many people have offered their prayers, warm thoughts and I know I have mentioned it before but I can't hardly express my gratitude to everyone. 

Some people have wondered why we have to go through what we do.  There is no easy answer.  It's just one of those things.  We were sent her to be tested, to see if we can truly become a disciple of Christ.  For some, that isn't always an acceptable answer.  There have been a few who suggested we just give up on Christ, get mad.  I personally don't feel like that is the answer.  I just know that by having faith and trusting that God has a bigger and better plan for us, we are blessed beyond measure.  Shelbie asked me the other day what she should say to people who think she should turn her back on God.  I told her she needed to figure that out on her own. 

A couple of nights ago after I was done playing the kids their bedtime songs on the piano and kissing them goodnight, Shelbie said to me, "I was thinking.  If I blame God for my problems then shouldn't I also blame God for the good things that happen and be mad that He is blessing me?"  That is a very profound thought!  If we turn our back on God when things are tough then how can we expect him to bless us when we haven't even tried to trust Him?  Whether we are facing horrible trials or blessings that overflow in our lives, both need to be approached with gratitude, faith and happiness.  I think I finally understand what it feels like to find peace in His rest.  When we yoke ourselves with Him, the burden really is lighter.  I don't know how it happens but it really does.  It takes work to maintain our place in His peace but it is so worth the effort. 

Tuesday, November 30, 2010

Pioneer Children

There is a song the kids use to sing when they were little in Primary at church.  It was called Pioneer Children.  I think that title is very fitting to use for the circumstance we find ourselves in. Just as the Pioneers walked long distances to create a better life for themselves and those generations to come, my kids are also part of an elite group of children living with SDS.  Allowing doctors to study them so that life will be better for those still to be diagnosed. 

 When I spoke to our doctor yesterday, he said the kids were in some ways, charting through new territories.  Of course lots of people have ITP and we are not the only ones with SDS and if it turns out the kids have mito disease, we won't be the first for that either.  The doctors are barely skimming the surfaces of these diseases as far as research goes.  Much has been learned but there are still so many missing pieces.  Unfortunately, we find ourselves stuck in that place where there is no sure treatment. We try one thing, hope for the best but if it doesn't work we move on to something else.  Everyday, is a day closer to finding those answers we long for.  For now, we just have to live the questions and the answers will find us in some distant day. 

It was interesting to listen to our Oncologist reason things out with me.  They aren't just planning for this week but for the future of my kids.  I was especially glad to hear that they are not only concerned with their physical well being but their emotional as well.

I guess as a parent and a mother, I too am learning to navigate through this barren land of chronic illness.  There are no markers or footsteps to follow of those gone before.  No defining borders and nothing to tell me I am on the right track. It can be a little overwhelming.   My kids too feel the weight of their responsibility to gracefully accept this life and find an element of 'normal' for themselves. 

I am proud of them everyday.  We have so much fun together despite the problems.  I can't express enough gratitude for all those who have offered prayers, visits, phone calls and warm thoughts.  That makes the journey all more peaceful.  We are really being watched over.

Monday, November 29, 2010

A Little Reprieve

Sunday ended up being a better day.  The headache was manageable and she had a couple of visitors as well.  It  perked her up and kept her spirits up.  One thing I can say about us, we still seem to find our humor even when things are pretty gloomy.  People hear what Shelbie is having to go through right now and I am sure they think the feeling here is pretty heavy and depressing.  It really isn't.  It's actually pretty upbeat and positive.  That's not to say we don't have our moments. Sometimes, I find myself wondering how the world can go on when we are stumbling so much.  It doesn't last long and hopefully, is normal. 

Today is pretty good so far as well.  Shelbie had signed up at the beginning of the month to work today and tomorrow.  She had a one hour project to do at Walmart for Crest, setting up a display and such and one to do tomorrow at Broulims.  Once you sign up for a project, it's pretty hard to get out of it.  We headed to the hospital first thing to get her daily CBC.  Her IV was not working properly so they had to pull it and stick her again. The poor girl is so beat up and bruised from blood tests and IV's.  Nurses are struggling to find a decent vein to use.  We got home after about an hour or so and she crashed until it was time to get ready for work.  She is still on pain meds and anti inflammatories for the headache so no driving for her.  I drove her to Walmart to complete her job at noon.

We have had a lot of little blessings and tender mercies already today.  Last night, when we were falling asleep, (I've been sleeping on her floor just in case she gets into trouble through the night) she said how much she just wanted a stuffed bear to hug while she is going through all this.  At the hospital this morning, the nurse brought in a great big stuffed monkey as a gift for Shelbie!  Not a bear but a really soft, cute monkey.  It was so kind of the nurses in short stay.  I have really been impressed with them this week.  I felt like that was an answer to prayers.  Even though it was small and insignificant really, I have always thought that if it's important to you, its important to God.  I pointed this out to Shelbie.  It was almost as if that monkey was a little heart message from God letting her know that he is near!  It's little things like that that truly make all the difference in this journey.

The second blessing we had was at her job.  The project today involved bringing boxes of Spin Brush toothbrushes out from the stock room and putting together a holiday display for Crest.  Oddly enough, Walmart had no stock left in the Spin Brushes! None on the shelves either.  The materials for the display were no where to be found.  Typically, if there isn't work to do then she is to stay for the allotted time straightening product or dusting the shelf of the product she is representing.  There was nothing to do so she was able to leave.    I was so glad.  I was torn this morning about calling the corporate office myself in California and cancelling for her but then I thought it would be important that we try to find our new 'normal' and get her back into a routine as much as possible.  I was hoping it would be a good distraction.  Just being in the store for 10 min was way too much for.  She was shaking and weak and ready to collapse.  Another tender mercy that by chance, there was nothing for her to do.  She will still get paid as if she did the job so another bonus for her.

The third blessing was that I was suppose to clean two houses today.  I cleaned the first one while Shelbie was napping this morning after the hospital.  The second one was suppose to start at noon, the same time Shelbie's was to start.  I didn't want to leave her alone at Walmart but didn't want to cancel cleaning either.  Just before we left, the lady I cleaned for called to tell me she had a kidney stone and wanted me to skip this week.  Whew!  I was relieved but sad that the lady has to feel so bad. 

I am really proud of the way Shelbie is handling things.  If you saw her today, other than being real pale, you would never know she had anything wrong with her.  I'm sure it makes people think we are making all this up!  I wish we were.  Oh, I guess I better mention the best part of the day...her platelets are at 72!  That is awesome.  I hope they continue to increase.  One thing about ITP is that it is very unpredictable.  One day it can be up, the next day rock bottom.  Our past experience with this, it has dragged on for months.  We don't want to go through this for months but if that is how it has to be then we are ready for it!  This will be a big week as we wait to see how long the IVIG will help the situation.  Though it is taking a huge toll on the rest of her body, it is nice to see that she is out of the danger zone for bleeding.  We will take whatever we can get at this point.  

Sunday, November 28, 2010

ID Tags

This week, being at the hospital so much, I have been intrigued with the nurses ID badges.  What is up with that?  No one, not one person showed any resemblance to their photo.  It was really weird, even the BYUI student paramedic we had last night.  His picture had him looking like a 90lb 15 year old but in reality he looked to be in his mid twenties and definitely not 90lbs. ha ha. 

Okay, just thought I'd throw that out there.  We had high hopes for yesterday, we really did but the headache kept getting worse throughout the day, the fever lingered and by evening the vomiting started.  Try throwing up when you already feel like your head is going to explode.   I've never done that myself but to watch Shelbie was less than entertaining.  My heart broke for her.  There was nothing more I could do and felt helpless so I took her up to the ER.  They started fluids immediately and morphine.  That helped her settle down and the pain eased up a bit.  She had two bags of fluids and a couple injections of morphine and sent us home with IV anti nausea meds.  The doctor felt admitting her could be beneficial but he wasn't opposed to letting her go home.  Shelbie chose to come home.  We were finished around 2am.  She has been sleeping well since then but I woke her up to take more pain meds at 7:00.  I'm hoping the pain doesn't get away from us.  They sent her home with the IV intact so she has an open line just in case.  I feel better about that. 

The excitement doesn't end there.  Just as we were walking out the door, Spencer was in an accident and his car is now undrivable.  He is okay but that was almost the straw to break my back.  Then I had to deal with attitude from my wasband....that was the last straw!

 As I walked with Shelbie into the emergency room, it felt like we had time traveled into the 1920's.  The snow was falling in slow motion from the illuminated night sky.  Across the parking lot, in the city park, a celebration was taking place.  The pavillion was festively decorated, people were bundled up and a tinny sounding Christmas carol played from the band.  I felt like I was stuck inside and old, old radio program.  The weirdest feeling ever.  I felt the whole world just spinning and nothing felt familar.  I turned to Shelbie and said, "Does this feel strange to you? The crazy sounding Christmas music, the onslaught of problems, coming to the hospital AGAIN? This can not be freakin real!"
She looked at me, even the way she looked at me seemed off, she stopped in her steps, shook her head and let out a grimacing chuckle, "Ya, your right, crazy".  I still hear that distant, old radio music playing in my head from the band in the park.

Saturday, November 27, 2010

Running out of snappy titles...

I am running out of snappy titles for these posts.  Suffice it to say, all the days lately are long, hard or tiring, disappointing and frustrating.  I have no idea what to call today's post, 'Headache from Hell'.  Shelbie-Sue was not lucky enough to avoid the worst headache of all time.  It isn't quite as bad as the last time she had IVIG but it's a 7 on the infamous pain scale.  Her platelets are coming up which is dandy.  She has a whopping 24 now!!  Not good at all but 23 better than 1!  Funny how what makes us happy shifts.  I use to be really happy with 100, even 50 didn't worry me too much but after living with 1 for a couple of days anything is a relief.

I am not enjoying the learning curve to all this.  Immune deficiency disease is not something I am very familar with at all.  It took me 18 years to get a handle on the ins and outs of the basic SDS disease.  I am part of an email support group and they have been helpful with information and ideas of what I need to be doing to keep her comfortable.  I still kind of exist in denial thinking this is just going to go away soon and everything will be back to normal.  The people who have SDS kids that also suffer with ITP do not make it sound like this is just a passing episode so it's a little scary.  They also can't believe she is at home and not in the hospital.   It's hard when doctors have the last say.  It is different looking at things from a professional point of view than the emotional and mental one that clouds our vision.  Somehow, you have to find a resolve, peace of mind that the doctor does know best.  I just wish I had more information.  We will now have to add Immunology to our list of specialists on board now.  Dr. Shimamura will set that up for us this coming May while we are there for bone marrow biopsies and mitochondrial clinic.  I would love to sit down with an Immunologist today and get my education started in that area so I can start asking intelligent questions and piecing the big picture together better.  Somehow, it all has to be connected.  Big sigh....

Friday, November 26, 2010

Super Long Day

Bad Photo, the room was really dark.  This is what IgG looks like.  The pharmaceutical company Grifols that manufactured this is in Spain but we have a Grifols plasma center in our very own little town, right on main street.  Funny they had to send it all the way from Spain!  God bless all those students who donate each day.  I wonder if they know they saved a life today?

Shelbie, not feeling on top of the world but graceful through it all.

The infusion from Shelbie's view.  Cool how you can see me sitting next to her in my pink, wool coat.

About 7 hours of infusion and we are done for today.  More tests tomorrow but that won't be a big deal, I hope.  The nurses today were terrific!  They were so happy, helpful and had a great sense of humor and treated Shelbie as if she were their own daughter.  That made all the difference in the world.  They took it nice and slow and loaded her up with benedryl and tylenol beforehand in hopes of avoiding the nasty side effects.  She spent all day with a low grade fever and pretty low blood pressure.  Not sure yet if those symptoms will prove to be a problem or not.  So far, no headache to speak of so we may not have to deal with aseptic meningitis. 

Her counts are still in the tank so she is far from out of the woods, especially the bleeding risks since she is still functioning on an insanely small number of platelets.  This is no immediate fix that's for sure but it's a start. 

Even though this last week has consumed my thoughts, still in the back of mind is the nagging reminder that mito tests are still pending and should be coming in days, maybe a week.  I don't imagine this white knuckle ride is over yet.  Until then, we will just keep hoping and praying.  I am really pleased with where we stand right now.  Of course it can always be better sometimes we have to take what we can get.

The fun begins soon...

Half an hour and Shelbie starts the IVIG infusion, unless by some miracle her body kicked into gear and is now making platelets hand over fist!  It could happen, it's happened before but only time will tell. 

Here are a few facts about IVIG
IVIG stands for Intravenous Immunoglobulin
It contains the antibody IgG that is extracted from the blood plasma
It can take up to 20,000 donors to get enough IgG for one treatment
Results can be seen in 24-48 hours though some may have to wait 3-4 weeks before seeing an improvement
Some may never see good results from this treatment.  After 3-4 sessions of infusion and no results, they
        abandon the course of treatment. 
If good results are seen, infusions can happen every week or every couple of weeks.
The cost of one treatment......$10,000.  Merry Christmas Shelbie!!  ( I'm just kidding of course)

This has been one expensive fall.  $40,000 for bone marrow biopsies and mitochondrial testing.  There is a shortage of platelets.  One article I read said they can be upwards of $5000 for one transfusion of platelets, more if it's irradiated.  Now a $10,000 bill for IVIG.  The cost of the hospital and doctors still remains to be seen.  Whew, this is not a cheap disease to have.  Hopefully, I won't get stuck with too much of this.  We should be covered but time will tell. 

Thursday, November 25, 2010

The latest

It's hard getting sick over the holidays.  Not much could happen today.  Tomorrow morning we will head back into the hospital for an IVIG treatment.  Hopefully she won't have too many reactions from it and maybe by some great miracle her platelets are coming back on their own and in my perfect world, we won't have to do the infusion.  It never hurts to keep hoping. 

Today has been quiet but filled with lots of well wishes and warm thoughts.  So many kind people extending invitations for me to join their families.  I was able to take a good long nap and felt much better about things afterwards.  I really needed some quiet time when I could just let the feelings come without trying to hide them or put on a facade that everything is okay.  I felt bad saying no to such kindness but it was the needful thing. 

I will update more tomorrow.  Things change quickly in our little crazy world.  You really can't predict what will happen next even though we like to try. 

Platelets

Platelets

Dripping away

One very tired, emotionally spent, super brave young lady. 

How do you spell INSANITY

I will tell you how to spell insanity; R -E -D  C- R -O -S -S.  I hate to give them a bad rap but they seriously messed up today!  This morning, Shelbie was suppose to get her platelets at 8:00am.  We arrived early, ready to deal.  The platelets had not arrived as anticipated because of the storm that has been in the area for a few days.  Salt Lake had grounded flights last night.  No big deal, she said they tracked the package with Red Cross and the platelets had been irradiated and they had been put on the first flight out this morning so it would just be a couple of hours.  We left, went home and waited for them to call us back up.  The nurse called around 11:00 to say that they didn't make the flight as planned but they would be put on a later flight but would arrive at the hospital at 2:30.  We went back up at 2:00 to get the IV started, bloodwork done etc.  2:30 came and we were anxious to get things started.  Her platelets had now dropped to 1000.  Very dangerous at this point for hemorhaging spontaneously.  We tried to be patient.  Just after 3:00 the nurses came in with some bad news.  The blood had arrived but it wasn't for Shelbie.  They had mixed up the blood!  So, back on the phone with Red Cross, they promise it will be on the next flight.  At this point, I thinking two things: I could have driven down there myself, drove it home and had her transfused long before now and how many next flights can there possibly be?  Finally, at 5:30 the platelets had arrived safely and were ready for her. 

It's really hard to describe everything I was feeling the moment the nurse hung the IV bag of platelets.  You would have thought we just received a heart or a liver! Something more precious than life itself.  I felt so much gratitude for the one person who has dedicated themselves to giving blood every month.  Such an easy thing to them I'm sure but to us, it literally saved our daughter's life.  Shelbie can't have the typical donated blood because she is a high risk patient and potential transplant recipient.  We can't afford for her to get Myelofibrosis, it would complicate and compromise a successful transplant.  I wanted to cry but didn't.  I was sort of in shock as I watched her parched body gulp up those precious cells.  It took less than 5 min for her to drain those honey colored platelets.  Amazing!  In a half hour, her platelet count had gone from 1 to 55.  A tremendous blessing!  Far from normal, but we'll take it!

So, here is the run down of what the next few hours hold for us.  At 8:00am this morning, (Thursday) we will go back to the hospital for another CBC.  If she has crashed again, then they will diagnose her with autoimmune thrombocytopenia, ITP.  This disease means that her immune system is seeing her platelets as a bad thing and destroying them faster than she can make them.  It is difficult to treat and there is no long term cure as far as I know.  If the platelet count is still at 55 or higher, then they will make the diagnosis of aplastic anemia which is essentially bone marrow failure.  She has been in bone marrow failure for some time but there are degrees of failure.  Remember from the results two weeks ago, the doctor said she didn't know how much longer their bodies can continue to compensate for their lack of marrow.  Well, maybe her body has finally had it.  Tomorrow will tell.  There is a third possibility that she has both in which case I really don't know what happens. 

What becomes the treatment?  If she has ITP, then we start infusions of IVIG which sucks!  When she had this three years ago, she contracted aseptic meningitis which is a rare side effect.  She would have to have this weekly and usually involves a day or two in the hospital if there are no side effects.  If it is bone marrow failure then we will keep her going with transfusions until everything is in place for a bone marrow transplant.  That could be months down the road.  It's a lot of information. 

All in all, I am overwhelmed with the outpouring of love and prayers.  A great friend came by tonight to cheer Shelbie up and we enjoyed just laughing and letting go of the heavy day.  Another person called to say she would donate blood for Shelbie, they have the same rare blood type!  How awesome is that?  I am really touched.  It will be a great Thanksgiving no matter what happens!  We are so blessed and live a life of abundance!

Wednesday, November 24, 2010

Who was I kidding? Ha, ha, ha!!!!

It's 11:35.  It's been a horribly long day with Shelbie in the hospital and waiting for Red Cross to find some intelligence.  Long story which I will report on next, in a different post entitled something like, "How do you spell INSANITY?"  Anyways, Shelbie is not out of the woods, not by a long shot and she is anxious about going to sleep so I will stay up through the night to be sure she doesn't have any bleeding issues, that means a lot of time to blog! I have to say, when I opened up my blog tonight, I realized that two days ago, I said how happy I was that this was going to be a quiet week "medically speaking" and was looking forward to a "happy, healthy" Thanksgiving.  How funny!!!  No really, it makes me laugh...you can't blame a girl for hoping!  Medically speaking, it has been chaotic, bizarre and so overwhelming I don't even think I am living in the same plane as the rest of you and I am so sick of speaking in medical terms I want to YAK! Anyhow...

For the last two weeks, I have been really thinking about the scripture in Matthew, Consider the lilies.  I realized that I can't change what is happening to us but if I have faith and believe then God will take care of us no matter what we face.  There is a beautiful song that shares the same subject and it's called Consider the Lilies.  Below are the lyrics.

Consider the lilies of the field,
How they grow, how they grow.
Consider the birds in the sky,
How they fly, how they fly.

He clothes the lilies of the field.
He feeds the birds in the sky.
And He will feed those who trust Him,
And guide them with His eye.

Consider the sheep of His fold,
How they follow where He leads.
Though the path may wind across the mountains,
He knows the meadows where they feed.

He clothes the lilies of the field.
He feeds the birds in the sky,
And He will feed those who trust Him,
And guide them with His eye.

Consider the sweet, tender children
Who must suffer on this earth.
The pains of all of them He carried
From the day of His birth.

He clothes the lilies of the field,
He feeds the lambs in His fold,
And He will heal those who trust Him,
And make their hearts as gold.

He clothes the lilies of the field,
He feeds the lambs in His fold,
And He will heal those who trust Him,
And make their hearts as gold.

Why is this important today?  Well, when we were finally able to leave the hospital tonight, the song playing on the radio was Consider the Lillies and the verse was the one "Consider the sweet, tender children who must suffer on this earth.  The pains of all of them He carried from the day of his birth."  I'm not going to lie, today has been so hard but insulating all this pain and sorrow is the fact that God is carrying us. He is carrying Shelbie, the boys and me.  All he asks of us is that we trust him and in return he will heal us and make our hearts as gold.  This shouldn't be hard; sometimes it is but what a return on a 100% safe, insured investment!

Tuesday, November 23, 2010

Really tough day

Today came at us from out of nowhere.  I had an early morning doctor appointment and when I came home, Shelbie was up and announced that she had lots of petichae.  Petichae is basically broken blood vessels, in Shelbie's case, happens when her platelets are low or dropping.  I wasn't alarmed or even really that concerned but thought with the holiday week, we should at least get a CBC just to be sure she wasn't in any danger.  We went with the boys to shovel the driveway of a widow and then drove over to the oncologist's office.  We have had a huge storm in the area for a few days now and we arrived just as they were closing down.  Most of their patients cancelled and they wanted to get the staff home before things got worse.  I told them it would just be a quick CBC and didn't even need to see the doctor since I can pretty much decipher what all the counts mean. 

We all sat in the waiting room placing bets on what her platelet count would be.  The winner had to buy lunch.  The guesses ranged from 115,000, 105,000 and 80,000.  Sam didn't want to play, I guess he was afraid he would win!  When they realized I said the winner had to buy lunch, Shelbie changed her guess to 2000.  Well, she was the winner.   What was suppose to be just a 10 min stop in the day, became a couple of hours of information overload.  Thankfully, we caught the doctor before he left for the day and we were able to conference call with Dr. Shimamura in Seattle. 

This is where we were three years ago.  Her counts were rock bottom; as they are today.  She ended up having chemotherapy for months.  Chemo is not an option this time.  After a lengthy discussion on what was causing the counts to crash, we all agreed that a platelet transfusion is what we will do.  That however, is not as easy as it sounds. 

She can only receive irradiated platelets.  Because she is a candidate for transplant, they have to remove any 'accidental' cells that get into the platelets from the donor.  They aren't a big deal for you and I, but can cause a lot of problems for an individual with Bone Marrow Failure Syndrome.  Not only that, instead of just taking any old pint of platelets from the bank which could be made up from many donors, they have to choose platelets that have come from only one source.  This means, that they need to find a donor who goes faithfully and regularly to give blood.  That sounds hard to me but apparently it is not as hard as it seems.  If they don't take these precautions and measures, then she could develop a secondary problem called Myelofibrosis. (I think I have the terminology right...don't quote me exactly though.  I will find out for sure tomorrow)  Essentially, this is when the bone marrow begins forming a spider web of scar tissue that could impede any chance of a successful transplant down the road, should one be needed. 

I am very grateful to these two great doctors who worked so well together and involved Shelbie and I in the decision making process.  It helped Shelbie feel a little more empowered.  Tomorrow morning, bright and early, Shelbie will receive a 'six-pack' of platelets at the hospital.  Throughout the day, they will check her blood and track what is happening to her new blood.  If the counts stay up all day, then the doctors will know that her drop in counts is caused from bone marrow failure.  If  by the evening, her counts have crashed again, then they know she has an autoimmune disease called Immune Thrombocytopenia or ITP.  I am glad that we are finally settling this debate once and for all.  Our Oncologist here believes that this is a case of ITP, I have always believed it is a case of bone marrow failure and have respectfully disagreed.  He knows I don't entirely agree and we have a good relationship about it still so that is good.  Neither one is something we want to have to deal with but at least knowing will allow the doctors to choose the right treatment for her instead of guessing. 

As you can imagine, this has been a really hard day to navigate.  I try to be positive and happy around the kids but tonight Shelbie said, "I'm sorry I am so upset.  Obviously this is no big deal, you don't even seemed bothered by it."  I explained to her that I can't just allow myself to fall apart or I would never put the pieces back together.  As soon I open the flood gate of sorrow and tears, it's anybody's guess if I will ever be able to turn it off.  I especially can't do that in front of my kids.  I am their anchor, their rock.  I have to present a strong front of hope and faith so that they have something to buoy them up.  I have all night, to let the tears escape and they are none the wiser that I spent the whole night trying to stitch my heart back together. 

Even though this has been a tough week, I still feel we have so much abundance, so much to be thankful for. God is good to us!

Sunday, November 21, 2010

Daring to hope

 With Thanksgiving this week, I am really hoping the kids stay healthy.  There are sick people all around us and it seems impossible that we might be so lucky as to dodge the strep or flu viruses but I am crossing my fingers and hoping.  Medically speaking, we should have a quiet week.  Tomorrow, I will spend a fair amount of time working with the National Shwachman Diamond Registry to get the kids, myself and extended family members registered.  They sent the large, overstuffed envelope of questions and procedures for me but it has to be filled out with a nurse from the registry so that will happen tomorrow.   I'm not exactly sure what being part of the registry means but it is something that our Oncologist deemed absolutely necessary so I suppose I will learn more tomorrow.  Other than one doctor appointment for Shelbie, Spencer and me, we have no other doctor visits planned. So, here's hoping for a healthy, happy holiday!

Thursday, November 18, 2010

If I didn't have bad luck...

Wow, what a day!  Tuesday, we spent the afternoon at the Ear, Nose, Throat doctor.  I believe, I would rather see a dentist for a root canal than have someone messing with my ears, nose or throat.  I had anxiety just watching what Shelbie and Spencer had to go through; air pushed through their ears, plier like things up their nose and all sorts of poking and prodding.  Yuk! 

Spencer was there because he is still having problems with his soft palette in his mouth feeling swollen and enlarged. Back in April, he sneezed and seconds later, his uvula and palette were resting on his tongue.  No one could figure out what was going on and as a wild guess, threw out allergies as the possible problem.  Spencer complains about it alot so I decided it was time for a specialist to take a look. The doctor wanted to do a scope while we were there.  They put a tube up his nose then dropped it down his throat.  I felt so bad for him.  It looked horribly uncomfortable and he takes after me with a deviated septum so it was really tight and uncomfortable.  From that, it was decided that Spencer was suffering with allergies so today, we went for allergy testing and a hearing test for both Shelbie and Spencer.

The allergist was really nice.  Spencer was tested for something like 56 different things from weeds to shrimp.  The list of what he is NOT allergic to is much shorter than what he IS allergic to.  He is not allergic to dogs, dust, molds,mites,cockroaches (what a relief!ha ha), cattle, horses, feathers, milk, eggs, wheat or soybeans.  Too bad we aren't farmers, he's not allergic to farming!  So, all trees, including pine trees he is allergic to as well as all weeds, and cats.  But, get this, the boy is allergic to POTATOES!  Seriously?  We live in Idaho and he is allergic to potatoes!! Not just a little allergy to potatoes but a moderate one meaning, he may be banned from potatoes!  He also has a sensitivity to peanuts!  I was shocked to say the least.  We have a follow up with the ENT next week but this might explain his constant congestion, runny nose, swollen palette, maybe even some of the nausea we deal with daily.   Honestly, I couldn't believe it.  Now I want to get Sam in there because its a fight to keep that kid from drowning in mucus on a daily basis. 

While the kids were getting their hearing tests, I decided I may as well have mine tested too.  I know I have hearing loss, I was diagnosed with that problem well over 10 years ago.  Lately, I have been feeling like I am underwater when I talk or sing and even play the piano.  I feel like my ears are plugged and it's been annoying the last year.  I was not expecting any news other than, 'you have hearing loss'.  No such luck!  My hearing loss is so bad I need a hearing aid.  NOT only that, the pattern of hearing loss suggests either trauma to my head when I was younger or a progressive bone disease in my middle ear!!!! How does this happen to me?! Now, I have to go see the ENT.  The Audiologist said that eventually, those ear bones will have to be replaced with a prothesis but I may have a few years before this has to happen.  Even with the surgery, I will still need a hearing aid because they can't recoup what I have already lost.  This sucks!

Sunday, November 14, 2010

The Giant Awakes

It is interesting to me how easily I am tricked into this false sense of security.  Like being lulled to sleep on a gently rocking boat.  How easily I try to forget all the issues that come with chronic illness in hopes of eliminating stress.  Not just try to forget but actually compartmentalize it all so that it disappears somewhere in the back of my mind.  For awhile, I really believe we are normal as the most normal family could be.  From out of nowhere, the boat isn't rocking so gently anymore and I can't believe I've been duped again!  Perhaps it's a good thing but it feels like ripping a bandaid from freshly formed road rash that covers my leg. It stings. 

This week seems to have lasted forever and existed before time.  Results are a distant memory until the tiniest thing wakes up this giant of anxiety in me.  Spencer called tonight.  He is sick.  The symptoms are vague, no energy, extreme fatigue,nausea, no fever, no sore throat, nothing that would make me think it is just the common cold being tossed around when winter sets in.  He doesn't think he can go to school tomorrow.  We make plans for him to stay home again and I say goodnight.  I will see him in the morning when he comes home from his dad's.  My own stomach is turning and my head hurts now.  Butterflies play another round robin in my chest.  Dread is all I feel when I think of having to call the school again to excuse him for another day.  I am reminded that even a cold can be a disasterous event in the life of an SDS child and I realize that maybe I am not as normal as I like to think I am.  Not many other moms panic at the first sign of a sniffle or sneeze!

A couple of days ago, I ran into the mom of one of Spencer's friends.  He is about a year or so in remission from Leukemia.  This week, he goes for his second CT scan in the year to make sure he is still cancer free.  Many of the things she feels, are not much different from my own worries and that is somehow comforting.  She knows the rising concern when her son complains of an ache or discomfort.  "Could the cancer be back?" She knows how it feels to anticipate a test and then the agonizing days that follow waiting for results. She knows the criticism from folks who think she has fragilized her son. She knows how to love him deeply each day because she has stared down death and won't let go without a fight.  She is not crazy, maybe I'm not either.

Friday, November 12, 2010

Silent Warriors

This has been a long week yet sped by at the same time.  Monday feels like forever ago.  The kids have been up and down with little viral annoyances or one thing or another most of which needed the attention of a doctor.  I took them all in on Wednesday afternoon.  Shelbie's platelets are running low again.  Not horribly low like we have seen in the past but they have dropped steadily since August, in fact dropped by 70,000 so now they are below normal.  I didn't really show much reaction while in the office but it was discouraging to hear. 

The following afternoon, when I completed my cleaning jobs, I headed to the library and ran some other errands.  It seemed that everywhere I went, I ran into other moms who have a child with cancer or who are going through chemo treatments with a child.  We know of each others struggles but don't really know each other.   We exchanged the international signal for mom's who take care of sick kids, a tender smile and a sympathetic nod and carried on our way. 

So much can be said in a smile and a nod to a mom who struggles with a child who is chronically ill. Without words, you know how they feel, you know of their sorrow, worries and anxieties.  You know the wearying battle they fight each day and yet, here we are doing what needs to be done and all with a smile on our face and no one is any wiser.  We are all Silent Warriors, a secret pact, hearts that are held together with these little moments when one and the battle they face is acknowledged.

Thursday, November 11, 2010

Baby Panda Sneezing

This is the cutest video.  I love how the mamma bear is just chillin' and then 'freaks' when her baby sneezes!  It's as if she is saying, "What the heck?!!"  SDS is like that sometimes,  things can be just fine and we are all chillin' and then out of the blue...someone sneezes!

Wednesday, November 10, 2010

Sufficient for the day

Yesterday, was a particularly difficult day and it sort of caught me off guard.  I guess after doing the mental and emotional calisthenics that we have been doing for an extended period of time, I was more exhausted than I realized.  I spent a lot of time just thinking about things and did some reading before going to bed at a very late hour. 

I read a passage in Matthew, a common phrase reminding me to 'consider the lillies'.  I've read that passage a million times, it has been a favorite since I was a teenager.  It starts out, "take no thought for your life, what ye shall eat, or what ye shall drink..for your Heavenly Father knoweth that ye have need for all these things."  It concludes by saying "take therefore no thought for the morrow; for the morrow shall take thought for the things of itself."  I really needed to be reminded of that.  I spent so much time worrying about a job, health insurance, financial issues, how I will pay for all these medical expenses, keep everyone happy and sane I was getting overwhelmed. 

I don't know how everything is going to work out.  I'm not sure how I will work and still take care of the kids.  I don't know how the bills will get paid today.  To look at the situation on paper, it is impossible and hopeless and if I worked 80 hours a week, it would still not be enough.  I need to do only what is sufficient for today.  The lillies don't do anything but stand there and grow.  The birds don't sow and the birds don't reap yet, they are taken care of.  Today, I am going to do only what needs to be done for today.  Though it includes a list of chores and errands, I will be glad to get the trivial things done that have been nagging me for months.  Manage what I can, let everything else go. 

Monday, November 8, 2010

Results

It's been an exhausting 31/2 weeks!  We finally got answers today!  Before I get to the results, I have a confession to make.  I wasn't going to say anything but told my kids today so now it's okay to blab it on a blog.  The Dr. called last Wednesday!  Ya, but I wasn't home.  Shelbie was though, looked at caller ID and it said 'Fred Hutchinson'.  She didn't know who Fred Hutchinson was so didn't answer it.  I checked caller ID and messages faithfully but I didn't recognize the name either.  I was so focused on looking for 'Seattle Children's' to show up.  Fred Hutchinson is the cancer hospital in Seattle where Dr. S does her research from!  For some reason, the message didn't show up until Saturday night.  She didn't give any results on the message, just that she needed to talk to me.  The rest of the weekend dragged and I only slept for 3 hours on Sunday night.  I was determined to swamp her with voicemails and messages until she called today. 
    
      On to results.  She said all three children have "empty bone marrow" meaning it is producing very few blood cells but there is no sign of leukemia.  The abnormal cells that have shown up recently are still there but they have not increased in numbers which is good that they are at least stable.  What does all this mean?  Well, we will have to be more attentive to getting their blood checked more often, every three to four months to be sure we are not seeing an increase in the abnormal cells.  She wants to recheck the marrow with another study as soon as the kids are out of school for the summer.  She is also beginning to search for a donor for each of the kids from the World Book.  The World Book is an international search that is done initially to see how many possible matches there are.  It is a service that is free.  When it comes time for transplant, then we will have to pay the big money to do all the detailed HLA testing/typing. 
    
      In about 4 weeks, we should have results back on the Mitochondrial testing so the waiting game begins again! Whew!  Regardless of the results, we will be going to Mito clinic in May when we return to Seattle for the next round of bone marrow biopsies.  Mito clinic will establish what testing needs to be done next if the DNA mito test comes back negative or we will be started on the Mito Cocktail if the test is positive. 
     
      She also requested that Shelbie and Spencer get some additional GI testing done as soon as we can.  It will help clarify things from where she stands.  We will have to iron out the process for getting all that done.  They are overdue to see their GI but that means a trip to Boise.  It may be something we can do here but more research needs to be done before that decision can be made. 

      At the end of the day, I am so relieved that we are not facing anything emergent.  It doesn't sound good, I know, but the alternative is worse.  Once the joy of this moment wanes, the reality is that their immune system is so depleted, even the smallest infection could potentially overcome them. She was surprised that they really are doing as well as they are considering how 'empty' the marrow is.  It is hopeful that for now, their bodies are compensating for the decreases.  I just pray they continue to do well but I know it can all change without notice.  For now, I choose not to even consider those possibilities. I know there have been many people praying for the kids and showing an increase in faith.  I know that those prayers have been answered.  Science can not explain how it is that my kids are not sickly when considering the state of their marrow and in and out of the hospital.  I know we are being watched over, there's just no other explanation.

     Of course the kids are relieved but it is hard to get too excited when they know that it was just one moment of victory.  There will be many more hurdles to jump and fights to win, that's just how it is with a disease that is chronic, rare and continues to have a mind of it's own.  Each one of those battles will be taken on as they come so tonight, they are guarded but happy!

Sunday, November 7, 2010

Wanna Help?

I know I have posted before about the Sullenger family.  I don't know them personally but have been interested in them since their only child died this past August.  They are in the middle of this awesome project to honor their daughter Preslee on her third birthday which would have been in December.  They are making tote bags for Primary Children's Medical Center PICU for parents who arrive in an emergency situation and need some essentials. You can visit the blog they have set up for this event here.  Once on their blog, you will see a list of things they are still in need of.  I just thought with the holidays just around the corner, you may be looking for a family service project to do and I think this is a great one!  Wanted to pass along a little cheer!

Saturday, November 6, 2010

Homeless Shelter





Last night we spent the afternoon and evening at The City of Refuge, a men's homeless shelter in Idaho Falls.  It was the best way to end a busy week.  I spent the day cooking and baking.  We arrived at 4:00 and got busy setting up chairs, filling water pitchers and getting all the food set out and warmed back up.  I was so proud of my kids!  They all jumped right in to work and didn't even have to be told what to do.  They even organized themselves for the assembly line of filling up the plates.  It was such a great feeling to be lost in helping other people.  We were all happy and enjoyed getting to know the director of the facility and some of the men who had been checked in for the week.  It is a very humble place, as you can tell from the pictures, they don't have much but boy, there was such a great feeling there.  God is watching over these people even though they are struggling through hard times. 

People started coming in around 5:30pm, dinner wasn't served until 6:00.  We couldn't feed anyone early because the director likes to start dinner with a prayer.  By 6:00, only a couple of chairs were empty and you could tell they were hungry!  There was a quite a mix of people, 4 women were there and the rest were men of all ages, the youngest one looked to be about 18 or 19.  One gentleman was excited to let us know that he had just gotten a construction job in Boise and would be moving there next week.  We spoke to another lady about her job doing laundry at the Shilo. Two men were completely deaf, didn't know American Sign Language so we were communicating with them in 'charade' form.  That was fun and I think they had fun with us too.  They kept coming up to the counter where we stood with big toothless grins and two 'thumbs up'!

The funniest part of the night came from Sam.  Bless his heart!  He kind of struggles sometimes when our usual schedule is altered so going down and being in a different atmosphere was a little difficult for him.  He was really stressed about putting potato chips on the plates.  He was nervous before it started and began feeling sick.  He asked me over and over again how to 'do' his assigned job.  I kept trying to reassure him that there was no way he could mess up.  Just before the prayer, he said to us, "Ahhhh! Ughh! this is so complicated, I know now why lunch ladies are so cranky everyday!"  When we got in the car, he said, "Well, that was fun but I know I don't want to be a lunch person when I grow up."

As we drove away, it was sad to see some of the people we had served walking alone on the dark streets, headed to who knows where.  I wished that I had a house a big enough to take them all home.  It was an awesome experience.  I want to thank my friend too, Kristi, who helped out with baking cookies to take, they were delicious and the guys loved them!  The kids want to go back again.  They think it would be cool to serve pizza because homeless people probably don't get to taste pizza too much.  I think we will save up to do just that!