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Showing posts from June, 2012

Heaven gets an Angel

We lost a little one to Shwachman Diamond Syndrome today.  His name was Zion and he was undergoing a marrow cell infusion.  His mom was the donor.  He has had a quite a battle the last few weeks but his body just wasn't able to handle the process of transplant. 

Only 50% of children with SDS who undergo transplant survive.  It's such a sad thing to come so close to relief from the bone marrow problems only to lose them.  Research is so important!  We are blessed to be hanging in there.  The longer the kids can get by, the better the odds of survival if the day comes that they do need a transplant.  Heaven forbid.  I am so grateful for the people who are spend so much time raising money for SDS.  The Butterfly Guild and Shwachman Diamond America are just two organizations that do so much good and my hats go off to them.   

Prayers go out to Zion and his family.  Thanks for fighting with us. 

Lice? NO!!!

The hives have become a huge problem and are seriously out of control. They have spread to his shoulders and arms. This morning, I had an early morning meeting so I woke Spencer up for work and then I left.   About 20 min after I left, he called in a huge panic.  I couldn't get him to calm down the pain and itching were getting the best of him.  I went straight home and took him down to the doctor.  Thankfully, they were slow and got him right in. 

At first the doctor thought it might be lice.  LICE?! Yes, duck lice. Are you kidding me?  He said there are a few rivers around here that have a lice problem especially mid summer and lice can cause the kind of rash that Spencer has on his stomach.  After a thorough check, he decided that it wasn't lice.  Thank my lucky stars! 

Given all the symptoms that Spencer is having in addition to the hives, the doctor thinks that his autonomic nervous system is just overwhelmed and it started with the biopsy last week.  So, the dysautonom…


Dysautonomia.  Something we have been dealing with for years and years.  It can be a problem for Shwachman Diamond kids and it is a mitochondrial disease.  At first my kids only showed symptoms of not being able to tolerate the heat.  Anytime they exert energy, they become incredibly flushed and worn right out.  Summer is hard because of the heat.  We don't have central air so sleeping becomes a problem when the house is 85 degrees.  Hence, our living room becomes the bedroom and all three kids crash on the floor and sofa since the bedrooms upstairs are probably 110 degrees. 

The past couple of years, they have had more symptoms.  Some have really baffled doctors but now that we are learning more about Mitochondrial disease I am starting to recognize how much this part of the disease has advanced.  One problem Shelbie and Spencer have had is hives.  A couple of years ago, Shelbie had hives non stop for almost 8 months.  We never did figure out what was causing them.  Shelbie gets…


I am so glad that last week is well behind us now.  The kids are bouncing back nicely with exception of a few glitches.  Spencer has had to use his rescue inhaler every 4 hours, every day since his biopsy.  I'm not sure what the deal is with that.  He hardly ever uses his rescue inhaler.  Maybe it was the anesthesia that just stirred things up a bit. 

Shelbie is feeling better today after a long weekend.  Part of me wonders if her body is getting use to these transfusions because they really don't seem to work anymore or at least as good as they use to.  The other side of me is afraid to find out what happens if we stop them.  I would feel awful if I said we were stopping and the adenovirus infection got out of control.  We have been so lucky this year to avoid any nasty viral or bacterial infections with any of the kids. 

The doctor told me that he would have cellularity results last Friday and asked me to call him to get them.  I consciously chose not to call him.  I need …

Moving on

Well, life goes on.  We haven't missed a beat.  Today, we are at the hospital again for Shelbie's marathon infusion.  It's going well so far.  She is sleeping which is good because she woke up sick with a bad cold and headache.  I worry that this just might end up being too much for her system.  Anesthesia, the biopsy and now a cold and new plasma.  I guess time will tell. 

Yesterday, the boys did so well.  They were in some pain of course but Spencer went back to work and managed to get through his shift.  It seemed like they were dropping things all over yesterday but no one can bend down to pick stuff up yet they kept asking each other for help.  Shelbie dropped a lid on the floor and asked Spencer to help her pick it up.  It was funny to watch those two try to get a lid off the floor.  I was not even two feet away and said, "Hey guys, I would love to help you out.  I am the only able body here today."
"Nope, we got this." and they did. 

Today, th…

Biopsies Done!

You know how when you have a really important day, you can't sleep the night before?  Last night was a disaster!  I went to bed at 10:30 knowing I had to get up at 4:30am.  By 1:30am, I was still laying there hoping to fall asleep.  I was exhausted but my brain just couldn't shut off. Sometime around 2:00 I must have dozed off, only to be awaken by the dog sitting on my stomach barking like mad.  I don't know what he was barking at but he was definitely in attack mode.  It took me a few minutes to settle him down. 

I fell back to sleep and at 3:30 a house behind mine and about 3 down had their motion detector light go off in the back of their house.  I thought someone had come and flipped on my bedroom light.  It was unbelievable how that light from so far away flooded my room and woke me up.  What do they think they are, a lighthouse?  Hello...we live in a desert!  I was so mad so I laid there, wide awake until my alarm went off at 4:30. 

Okay, so back to the point of m…

Only hours left

Tomorrow morning, bright and early, as in 6am bright and early, the kids will have their bone marrow biopsies.  Hopefully, by 10:30 or 11:00, the last one will be coming out of recovery and joining the other two. They are already arguing about who has to go first.

We've already been to the hospital to fill out the mountain of paperwork and each of the kids had to be interviewed by a nurse.  At first, I was a little bugged because the nurses usually just let me answer all the questions like family history, medications, symptoms, why the procedure is being done, drug allergies etc.  This time, three different nurses took a kid to have them answer the questions.  I still think that was not the best thing to do on their part because how do they know the kids have one clue about what is going on?  It's not like we are coming in for a tonsillectomy or something, this is complicated.  I went with Sam and I noticed that our interview took 20 min, and Spencer and Shelbie were standing…

Facts of Life

The past year has been one of the hardest I would say as far as the ups and downs.  It hasn't been just one thing that has made it hard but the combination of lots of things.  There have seriously been moments when I literally felt crazy and the term 'nervous breakdown' felt really close to home.  I not one to stay down or depressed for too long but admittedly, it's been harder to pick myself up this year.  I must be getting old.  I blame getting old on everything.

This past week I have mostly been trying not to care.  To trick myself into just not giving a hoot anymore about anything and just let life steamroll over me.  That was working pretty good, or so I told myself.  I have tried every other method to dig out of this funk but nothing else was helping.   I heard something that changed my mind.  It was a portion of a talk by Jeffery Holland he gave to some missionaries in the MTC.  Here is what I read:

"He then went on to explain why missions and life have to …


45!  The number of bone marrow biopsies I have sat through.  I have watched all but 9!  I can hardly believe it.  Today, our Oncologist called with the bone marrow schedule.  Next Monday, the fun begins bright and early at 7:00am.  Not fun really, in fact, you would think that after this many, it would be second nature and no big deal but let me tell you about my stomach...

It's been in knots and turned inside out for most of the day.  I hate the anticipation of it.  We are late in getting this scheduled and that has bothered me yet at the same time, there was no way I could have done it sooner.  We are suppose to do them every 6 months because they have all had abnormalities show up and we have to watch it closely to be sure it isn't turning into Myelodysplasia or Leukemia.

This Wednesday, we will spend a large part of the day at the Oncologist's getting pre op stuff done.  Because of the mito involvement, we have to change up the anesthesia protocol and I'm hoping t…

They're all mine!

I follow a few blogs of people who are facing and overcoming huge health obstacles.  I have learned one thing, never underestimate the trials God can bless you with.  Tonight, my heart is aching for a few people who are fighting the fight of their life. 

I have been following one couple for about three years.  She has CF and three years ago, underwent a double lung transplant shortly after giving birth to their daughter who was more than three months premature.  She developed lymphoma shortly after receiving her new lungs and had to start chemotherapy treatments.  She began to thrive, their daughter survived and they have been doing well.  A couple of months ago, she went into rejection out of the blue.  She is now fighting for her life and the prognosis is grim.  Her oxygen, lung function and weight are all worse than pre-transplant.  Aside from their daughter they have taken in several foster children as well. Their faith is strong and they inspire me with their belief and trust in …

It's to be expected

I can't even describe how strange life has been over the past, I don't know how many feels like it's been months actually since I felt normal.  Tired doesn't even come close to describing how I feel, nor does exhausted.  It's something far heavier than that.  It feels like I just keep tripping through each day, hoping at some point, I either fall so hard it is physically impossible to stand up or I actually get a break sometime sooner than later. 

Somehow, some way, I just keep on moving.  I look around and see perfect people, their perfect life, their perfect happy, healthy family and even that makes me tired and it zaps me even more.  The kids are struggling more and more everyday and I am not making a very good shock absorber.  We are all so tired.  More than once this week, one of the kids has asked when we will just be able to have one week when nothing breaks down, no one is sick, we don't have money problems and we could actually do something…