Thursday, June 28, 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. 
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Wednesday, June 27, 2012

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 dysautonomia is causing all these problems.  He started Spencer on Prednisone to try to calm things down.  Oh, how I hate prednisone!  Remember last November when Spencer took prednisone...it led to a week in the hospital with malignant vomiting. 

He was able to sleep most of the afternoon with a little relief but I have maxed him out on Benedryl, Ranitadine, and Hydroxyzine, all suppose to give relief to the itching but they work for about 2 hours and then he's climbing the walls.  He is begging me to take him to the ER for an IV med that will work better but I'm not sure what would work any better than what we have.

It's crazy how fast things can go from bad to worse in just a matter of hours, sometimes even minutes.  Hopefully, things will settle down later tonight and I am on my way to the store because Spencer now has the munchies like crazy!  Thank you Prednisone for ruining my grocery budget!
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Tuesday, June 26, 2012

Dysautonomia

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 hives anytime her skin has pressure against it like when they do a blood draw or start an IV.  Even taking a band aid off will trigger a week of hives. 

Now it's Spencer's turn.  This in addition to his ongoing asthma attacks that we are still dealing with.  Here is an unbelievable picture of his hives.  Just remember, this is not a sunburn.  They are raised hives.


It started on Saturday night when he went bridge jumping with friends.  They showed up after the first jump.  Monday morning when he woke up, they were down quite a bit and his stomach was almost back to his normal, pale colored skin.  After his shower, they came back.  They come and almost go.  It seems like anytime water hits his skin, he breaks out in hives.  Monday, he went bridge jumping again and they were worse.  I am pretty sure he isn't allergic to water but the impact of the water hitting his skin is causing the hives and rash.

I think the 'trauma' of bone marrow biopsies, triggered a flare in his dysautonomia which also explains the asthma attacks.  I have been doing tons of reading tonight to try to figure him out.  I could take him to the doctor but they won't know what to do either. 

As I read tonight, I found an article that said dysautonomia can also cause vision loss.  This totally makes sense for Sam.  The last few months he has been losing his vision in his left eye.  Completely gone.  It's kind of scary.  It comes back after about a half hour. 

I never thought that 20 years later, I would still be learning new things about this disease.  I find the human body so incredibly intriguing.  I really wish I was a doctor because then I might actually know how to take care of them.  Until I get my degree, I hope these problems with Spencer go away soon.  He is most uncomfortable.  Just in case you wanted to know more, go here.
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Monday, June 25, 2012

Waiting

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 to be ready to hear the results and I wasn't on Friday.  I wasn't today and I probably won't be tomorrow.  8 months ago, when they had their last biopsy, the amount of cells in the bone marrow were sparse; 20% on average for all three of them.  In fact, Dr. Shimamura's words were, "Their marrow is empty." 

I don't understand how their peripheral blood counts can look okay, not normal by any means but they hang in there yet there are hardly cells being produced.  I don't know how that happens.  It sort of gives me a false sense of hope.  I am expecting 'empty' again but hope it hasn't dropped below 15% and in the territory of Aplastic Anemia.  That would suck. 

You know what else would suck? If those abnormal cells that have been hanging around have progressed towards Myelodysplasia or Leukemia.  I guess the more I think about, the more I like waiting in la la land where ignorance is BLISS.
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Wednesday, June 20, 2012

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, the boys are not doing as well.  Spencer went to work this morning and Sam came with Shelbie and I to the hospital.  Spencer came up after work for lunch but he was sick when he got here.  After getting some food on his stomach, he seemed to perk up a bit but then the strangest thing happened to Sam. 

When we got back to Shelbie's room, Sam said, "Mom, I can't see my hand!"  He was waving it right in front of his face!  Ughhh!  Not cool.  There isn't much I can do since I am tethered to this hospital chair but start calling doctors to figure out what is going on and get an appointment for him. 

It's so strange to me how these events aren't even really surprising.  Well, surprising, sometimes but we somehow just fit it into the workings of the day rather than having it become an event.
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Monday, June 18, 2012

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 my post.  Things started out pretty good.  The nurses were great!  Their nurse was a friend of mine from college days and she was so good.  I loved the two anesthesiologists we had.  Both are new and I will definitely request them again.  They actually called the Docs in Seattle like I had asked them to do. 

We ran into a few snags when it was Shelbie's turn.  Since she was fasting and hadn't taken her morning medications, she was getting pretty sick with acid reflux.  So bad in fact, she was having a hard time breathing and was in a bit of pain.  The anesthesiologist was concerned about this since they were only going to use a mask not IV meds.  He called Seattle again and they decided the risk of using Propofol was lower than the risk of using the mask and losing her airway or having her aspirate the acid into her lungs.  So, Shelbie had to be intubated.  That created a few unexpected problems and she was in recovery a long time until they could get things stable. 

Shelbie's bone marrow procedure took the longest too.  He had a hard time getting a good sized core of bone out.  A small vein was knicked during Spencer's biopsy so he has a little more bleeding than usual.  He is also having more pain than he has before.  Sam hasn't even asked for pain medication since we got home.  Other than really sore, barky throats and a sore back for all of them, in differing degrees, things could be better but they could also be worse.
Sam wearing his Bair Paw.  This is a really cool gown they use in the surgery department.  A hose hooks up to the gown which blows either warm air or cold air.  They use it to warm up a patient after surgery or cool them down. 

Cool Bair Paw socks
Shelbie was pretty nervous and wasn't feeling well.  Of all the kids, she is getting worn out with health problems and I notice that things that she use to handle okay, just overwhelm her easily.  It makes my heart break.

Spencer taking things in stride.  He was putting together a theme song for each of them that he played as they were being wheeled to the O.R Suite.  For Sam, he played the song, "The Final Countdown"  His song was called, "Don't wake me up" and I can't remember Shelbie's song.  Spencer has a knack for offering up humor at a time when no one can muster it on their own.  That's talent!

Spencer on Versed.  Still has that mischievous smile.

I hate this view!!!  It's the worst part of any procedure...letting them go into the hands of someone other than me. 
 I am glad this is done.  Right now, I am exhausted and really haven't thought about what the results could be.  I am hoping for stable right now and no increase in cell abnormalities.  I have heard that kids on the Mito cocktail actually see improvements...sometimes.  That would be nice to hear for a change. 

I have a million things to do before Wednesday but I am having a really hard time getting focused and switching gears.  The kids have been watching movies all day and I suspect tonight will be more of the same.  The house is a disaster but who cares?
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Sunday, June 17, 2012

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 around after about 5 min. 

When I got through, I went back over the information they collected from Shelbie and Spencer and filled in the blanks.  They got a lot of information right but they answered 'NO' to questions like, "Do you have any Neurological problems?  Do you have breathing or respiration problems? Do you have blood disorders?" 

Hello kids...Does Mitochondrial disease ring a bell...a huge neurological disease or how about Shwachman Diamond Syndrome, bone marrow failure, neutropenia...those little blood disorders?  The reason we are here in the first place?  How about Asthma?  Spencer's swollen palate? You didn't think that information was important?  We got it straightened around and by the time we left, the nurses eyes had glazed over and they were slightly overwhelmed.  If they thought that was bad, wait til Monday when things get really hairy!  Spencer brought his friend with us and we about had to peel her off the floor and drag her out of there.  She didn't know Spencer was sick and it was a little too much information for her.   I am glad however, that the kids are showing some initiative in taking care of themselves. 

I usually get the kids a little bag of goodies to open when they come out anesthesia.  Last week, Sam said, "Mom, you know how you get us a present for having our bone marrow biopsies?" 
"Ya."
"Well, I was wondering if this time, I could get a long board?"
"Does it fit in a gift bag?"
"I don't think so." he said
"Hmmmm, then no."  I replied with undertones of chuckling.
He walked off shaking his head and muttering to himself, "It was worth a try." 
Ha, ha, ha...silly kid.

Tomorrow afternoon I have a job interview.  Nothing like adding another stressful element to an already nail biting kind of day.  I really hope we are home and the kids are sufficiently knocked out on pain meds so they won't even notice I am gone.  I hope I can change gears from medical to website design and writing.   Wednesday, we are back at the hospital for IVIG so it will be a crazy kind of week, the kind I hope we survive without too many psychological tics by the end of it all. 
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Thursday, June 14, 2012

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 be hard. He explained about the purpose of trials more clearly than I’ve ever heard it explained before. He said that missions and life were never meant to be easy. It has to be hard because salvation is not a cheap experience.

 He said if we are going to be His disciples, we have to have something to show for it. We have to have evidence of our devotion to Him. We have to have evidence that we desire to be like Him.

 He explained that Christ chose to retain the wounds in his hands, feet and side so that they could be the symbols of his messianic mission. Everything else about Him is perfect. His wounds are evidence that there is sacrifice involved in serving the Lord. They are there to remind us that it takes suffering, heartache, anguish and pain to become like Him. There is no other way. Throughout our lives, we have to have times where we cry in our own personal Garden of Gethsemane. We have to have times in our life where we take a step or two to Calvary.

 Elder Holland continued by very emotionally saying, "if you are going to be his disciple, how dare you ask, how dare you not go even close to a fraction of the cup He drank. How dare you ask to never go close to the tears He shed and the pain He felt. There is suffering for the saints."

 I guess all the tears are not wasted.  I love that he said, "it takes suffering, heartache, anguish and pain to become like Him.  That's exciting!! I guess we are right on track!  Who knew?  I have felt all of those things daily, multiple times a day and here I thought I was weak and depressed but no, it's part of the battle!   I have to stop fighting against the current of trials.  I have to get back to accepting them for what they are.  They are more than a heartache, more than a pain in the neck, my life is depending on getting through these events and setbacks with more grace than I have been mustering this year.  I now understand that the struggle is not something to feel guilty about but it is part of the process. 
It's strange to me how just a shift in thinking can make such a difference; (a little sleep and pain relief helps too cause this arthritis is killing me!)  Not only did I read this passage but I have heard the exact same excerpt on the radio twice this week.  It was obviously something I needed to hear and I am glad for the course correction. 
So, does it take away the worry, the anxiety, the fear, the anger, the resentment all the other hard and ugly feelings?  Not really.  It has softened my heart a little bit and given me a new sense of energy to get through the next few weeks.   With the help of this and a few very distant friends I have never met before but along the cyber highway of chronic illness,  I feel ready for the bone marrow biopsies and ready for whatever news they hold for us. Even after we get through the actual procedure, it will be days and days of waiting impatiently patiently for the results. Here's to enjoying the ups and downs.  I really hope I can hold on to the resolve I have felt today.



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Monday, June 11, 2012

45

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 they get it right this time unlike Sam's surgery a couple of months ago. So, numbers 46, 47, and 48 are just around the corner.  It almost seems impossible we that have done so many of these.




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Sunday, June 10, 2012

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 God.  I feel a strange connection to this girl.  She reminds me so much of my best friend growing up who passed away from CF.

I follow a young mother with Melanoma...ohhh, I'm so glad that isn't my trial.  She is strong and a fighter but what a fight!  I have a friend who's son has been fighting sarcoma for over two years.  It started in his hip and spread to his lung and heart.  So many times, I thought it was over but last week, he had his cancerous lung removed and the cancerous portion on his heart and he is leaving the hospital today and for now, there is no evidence of disease in his body. 

I believe that we are all given the trials tailored to our needs.  The trials that will stretch us and help us grow.  Some people seem to have more than their share but somewhere in the breaking heart, is a space reserved for a special kind of strength.  A strength, though sometimes small, is just enough to keep that fighting spirit that helps us pick ourselves back up and remain steadfast on the battlefront, in the throws of the test.  I am glad for my trials and after reading about my blogging friends, I will gladly take what we have.  They're all mine and I wouldn't trade them with anyone.


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Friday, June 8, 2012

It's to be expected

I can't even describe how strange life has been over the past, I don't know how many weeks...it 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 fun and restful and pretend we have a life like the people around us who are off having fun for the summer.  Who knows.

I started physical therapy this week.  I really need to find some relief for all this pain I am constantly in.  Never before have I felt crippled until the last couple of months.  If I sit for more than 10 min, I can hardly get moving again and walk like a gimp til the blood gets flowin again.  If there is air conditioning going then my limit drops to about 5 min.  The PT guy thinks that strengthening my core muscles will help with the pain, I hope he's right!  I will need to start wearing a brace when doing things that irritate my back and cause pain like housecleaning and sitting for long periods of time.  I think I will pretty much be able to hide the brace so I won't draw attention to my lameness.  Basically, he said his goal is to help get the pain under control but there is nothing he can do to reverse the damage or even prevent more damage.  It's just an unfortunate thing.  I'll say.  The best part of PT three times a week is the 20 min. massage he gives me...ahhh.

I have been totally slacking on getting the kids bone marrow biopsies scheduled.  Even our doctor in Seattle is calling, wondering why I am dragging my feet on this.  I just don't think I can handle anymore bad news which makes no sense at all.  Not doing the test isn't going to stop bad things from happening.  My goal is to get this done by the week of the 17th.  Shelbie will probably have her next infusion right around then and that might work out well since she will be so sore after the procedure, she will just want to sleep anyways. The good thing is, we won't have to travel but a couple of blocks.

I do want to clarify, there are still good things happening in our lives and I can't deny that we are blessed in many ways.  Despite the difficult moments here and there, we have super happy moments too.  At the end of the day, nothing beats the feeling of being together and having each other to rely on.  I have come to the conclusion that tough times like this are to be expected and I know it won't last forever.  More on that another time.


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