Wednesday, June 26, 2013

Missing Pieces

Well, as promised, more on Shelbie because I know you are just sitting on the edge of your seat waiting to hear the latest and greatest in the life of us crazies!  Are you sitting down?

Shelbie doesn't have Vasculitis!  YAY!!!  Shelbie has Ehlers-Danlo Syndrome!  BOO!!!  What is that you ask?  Well, that explains her chronic and sometimes debilitating pain.  Ehlers-Danlo is a genetic disease that involves abnormal collagen.  The specific name of the spectrum that Shelbie has is Hypermobility Joint Disorder. The short explanation is that she has loose joints and they extend farther than they are suppose to. Because this is a collagen issue, it affects her skin, muscles, tendons, ligaments, joints and blood vessels and a host of other things.  It sounds benign enough and simple...but it's pretty complex and actually a bit scary.  Because of the involvement with her vessels, she is at high risk for strokes and aneurysms.

I am also in the middle of discovering if this is somehow linked to the abnormal telomeres.  Telomeres are responsible for premature aging.  They protect the chromosomes and chromosomes can only replicate so many times before they wear out...hence the aging process.  By the time this happens, theoretically a person is 80 or 90 years old and death comes.  Part of the aging process is the slowing down of collagen that our body produces.  I am researching if there is any link between a collagen disorder, which we have now confirmed and short telomeres.  I may come up empty handed but its a place to start.

This was huge news yesterday.  It explains so much!  I also feel really bad because I have often forced Shelbie to do things that hurt her.  I wasn't as sympathetic as I should have been when we were in New York for Spencer's wish and she complained and carried on that she was in so much pain.  I judged her, misjudged her.  The doctor told me that this condition is extremely painful and it will be a lifetime of chronic pain.  When we have to be doing strenuous things, there's a big chance that Shelbie will need to have a wheelchair, like walking around NY City.

I remember a few years ago, we were at my sister's in Edmonton and the whole family was there.  Everyone wanted to go to the West Edmonton Mall, a gigantic mall.  Everyone scattered and we planned to meet up for lunch.  It took the kids and I close to two hours to walk past about 20 stores.  Shelbie had to keep stopping because of pain and weakness.  I totally lost my cool.  I may have even said some things to her I regretted later.  Now, all those times I was inpatient are in the front of my mind and I feel horrible.

I have never wanted disease to define my kids so I push them.  Yes, we have a unique set of issues and illness but we have all sorts of blessings, hopes and dreams too.  No one ever dreams of being sick.  I never hoped or dreamed of having sick kids and the last thing I want to witness is my nearly 21 year old daughter in a wheelchair. I guess I've been too hard on her, discounting her pain, her fears and her feelings.  It's really tough to find a balance between what she can and can not do without giving in or giving up.  I guess this is all part of the process.  Something we will need to feel our way through.

There was one funny moment yesterday.  The doctor was having her do 9 physical things.  With each thing she did, she did it so well!  Inside, I was cheering her on...'You can do it Shelbie...show this doctor you aren't sick!'.  When she was successful, which was 7 of the 9 tasks, I said, "Yay! Great job!"  It was a proud moment.  Well, it was a proud moment until I found out it was a test for all the abnormalities she has!  The more she passed the more extreme this disease is.  Sad.  I guess I'll think twice next time my kids appear to be doing so good!

Well, that's it for now.  My mind is a bit of a tornado right now.  So much to process.  I posted to my SDS group if anyone was familiar with this disease and an impressive number of moms came forward saying their child was recently diagnosed with this as well.  It's nice to know we are not alone.  There's at least 5 or 6 of us with identical problems!  That's a lot in the world of orphan diseases, really!

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Tuesday, June 25, 2013

Last week's review

It's been an interesting week for sure.  In the past week and a half, Spencer has had 3 major Asthma attacks.  Each one just as bad as the first one.  I have no clue what is going on other than our faith is just being tested right now.   He left Sunday on a trip with his dad so hopefully, he manages things better this week.  I'm glad he gets a few days off from work and can hopefully get a little rest and re-fuel.

Sam hasn't had that great of a week either.  He has had a lot of problems with losing his vision.  He plays hard and goes hours without stopping.  By the time he gets home, he is wiped out and I think is body just gives out and his eyes seem to the be the way that fatigue is manifested.  This week, the vision loss has been followed by extreme pain.  Pain bad enough that even he admits he needs help.  Usually Sam just ignores his discomforts and chooses to live in denial.

He is headed for a huge Scout Encampment.  They are expecting 11,000 boys to celebrate the anniversary of the Scouting program.  He will leave tomorrow and the weird thing is, the camp is in the middle of the desert.  Usually, they camp by a river or some water source.  It's unfortunate for Sam that they aren't.  His dysautonomia makes him overheat so fast.  Heat stroke is something we have to deal with almost every day or at least several times a week in the summer.  He is pretty nervous about his eyes and his temperature when he goes.  He is only 40 minutes away so I feel alright letting him go, knowing I can reach him in a half hour if necessary.

Spencer and I had a chance to talk about the future and the problems with his marrow.  He could stay home from his mission and begin Plasma transfusions to add some support to his crumbling immune system, or he could continue with his plans and leave things up to the Lord.  I am fine either way.  Actually, I think his life will be more enriched going than staying but it's a decision I want him to arrive at.  Without even thinking about it, he said, "No, I'm going.  I will be able to manage.  I knew it was going to be like this, a hundred road blocks for me to get over before I left.  I have to trust that everything is going to be okay."

That's my boy.  Trusting in the Lord.  It doesn't mean there isn't an underpinning of fear and hesitation to some degree.  There is.

As for Miss Shelbie...we added another piece to the medical mystery puzzle today.  More on that to come.

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Thursday, June 20, 2013

Deep Breath

This has been an all around busy week.  Crazy busy!  I had to work 14 hours straight yesterday and when I was done working, I got started on my new project; a spreadsheet of all the kids symptoms and a list of all the possible diseases and complications.  It is interesting to see this all come together.  I took it in to our doctor today and he pointed out how the majority of problems are genetic, not just random things that seem to happen to us.  I'm not done but glad I have undertaken this massive job.  On top of all this...I started a certification course on healing environments.  As if I needed one more thing to do!

As I said,  I met with our local Oncologist to go over the bone marrow biopsies from Seattle.  Before we started, I let him know what I thought of doctors who don't communicate well; who try to sugar coat things instead of being honest and just telling it like it is.  I know he didn't have anything do to with the latest debacle but still...he represents a group of people who are really starting to get on my nerves.  It took my complaints graciously and agreed they have some collective work to do!

I was pretty much right about my interpretation of the biopsies.  They are not favorable at all.  He was really good to explain in detail what all the big words meant and even drew me some diagrams to help me understand some of the problems.  Like, Shelbie's report showed a deletion of chromosome 11 in her white blood cells.  There isn't anything we can do about it for now but still very important information.

His take on the telomere problem was interesting too.  He did confirm that they are short on both boys and has a hunch that they will continue to deteriorate until they are in the danger zone like Shelbie's are.  Telomeres protect your chromosomes from destruction.

When it comes to Spencer's left shift, he believes that he is now entering the twilight zone that Shelbie has been in for the past three years with her immune system failure.  Spencer got a bad virus back in October and he is clearly still trying to recover from it but his marrow just isn't strong enough.  It is now only functioning between 10 and 20%.  His peripheral blood is showing signs of straining as well.  It is calling up soldiers from anywhere he can get to fight the infection he has, even cells that don't typically fight disease are being asked to fight.  It shows the desperate situation he is facing.

Samuel is also in the same sad place.

So, what do we do now?  Well, if Spencer wasn't leaving in 5 weeks, he would be starting IVIG.  We would only have time to do one treatment and then he wouldn't be here for monitoring so that isn't going to work.  We could try an antiviral but the side effects are horrible and could be like throwing gasoline on a fire.

For Sam, we will start back with monthly blood draws to make sure he isn't getting worse and then we will likely repeat the bone marrow biopsy in 6 months.   For Spencer, we have decided to do some less invasive things in hopes that his body will be able to catch up.  The doctor also said, we need to eliminate stress from his life, have him get 9-10 hours of sleep a night, eat plenty of non-inflammatory foods and exercise.   HA, eliminate stress....ya right!  We will try.

In the meantime, it is looking more like Shelbie and Spencer will start treatment for Vasculitis, an auto immune disease.  We will confirm that in the next week.  Their immune system is attacking their veins and arteries.  This explains the petechiae, hemosiderins, carotid artery murmer and the fainting spells.

I will leave it at this for now.  There was a lot more but I still need to digest it.  I feel a little more settled after visiting with our doctor.  It helps to leave the kids behind so I could just get all my worries out there instead of trying to talk around them and sugar coat things so they don't get worried and stressed.

Sometimes, its just validation you need in order to get through another week.

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Sunday, June 16, 2013

Ahh...Sunday

So glad it's Sunday.  Sunday seems to be my saving grace.  The day when I actually feel like I can take a big deep breath, big enough to get me through another nail biting week.

I am grateful today for the people God places in my life to help me when things are tough.  This whole week has just worn me down.  Not only am I a poor communicator, our life feels so big and so overwhelming at times I just keep to myself because I know any kind of emotion I show will just be misconstrued as me losing faith or being depressed.  I'm not doing either, I'm just tired.

I am glad for my friend today who, with her talent and skill in the medical field was able to help me sort through some the bone marrow results and provide me with more knowledge than just Wiki and other internet medical sites. She is a very smart, single mom and we have a lot in common.  I appreciated her as we talked about how hard it is to let your sons leave on missions but even harder when they are not the healthiest specimen out there. Just getting some things out of my head helped me clarify how I will ever survive the next two years without Spencer.

I realized that I am feeling a bit of grief.  My whole life as been defined as a caregiver.  When I am not taking care of my kids, I am taking care of other people's kids or my elderly friends.  I have cherished this role.  It has definitely added a dimension to my life that I wouldn't have any other way.  Caring for others is my favorite job in the world.  I pray that I will be healthy enough so that I can continue to do what I love.

I think I am feeling a little sadness knowing that my role in caring for Spencer physically is winding down.  I'm sure there will be some things I will need to do but for the next two years, he will have to take care of himself, relying on the Spirit to guide him as he takes care of his own needs.  I will be turning Spencer's physical care over to God but I can still care for his Spirit.  I am excited to be able to communicate with Spencer in a different way now, helping him grow in the Gospel as he serves the people of Colorado.  This is going to be a learning curve for me as we enter this new phase of adulthood, chronic illness and letting go.

Letting go is going to be hard but I know that through the process of doing that, greater blessings of happiness await us.  I know I've said it before but we just have to stay in the moment.  Thinking about too many other things only proves to feed the despair I am sometimes prone to resort to if I'm not careful.

Ahhh....Sunday!



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Saturday, June 15, 2013

A little Faith and a lot of Prayer

This week as been a gem.  A real gem.

I will readily admit that I could have handled things differently maybe but then again, maybe my reactions to the much different written bone marrow biopsy report than the verbal one was warranted.  I don't like surprises.  I don't even really like good surprises.

I guess the good thing is, I only allowed myself to be upset for a few hours, not a few days.  I haven't hid myself away in the back of the closet in the fetal position crying over the news nor have I spent endless hours in my pajamas eating cream puffs and bon bons. (Well, I did discover Lindor truffles and now I have a hoarding addiction to them, but it's not the same as depression, its not.  They do take away the sting of this mess when the dark chocolate outside is soft from the heat and the inside is melty goodness, then you just bite a tiny whole in the outer part and let the melted inside run slowly down your throat, bathing every taste bud in a healing balm of premium dark chocolate....hmmm, mmm anyways...)

Anyways, I'm over it.  Kind of.  The worst part of all this is that my kids aren't 5 year old, computer illiterate beings anymore.  They are grown and they are smart and they want to be proactive.  They want to know the details of their bone marrow biopsy reports.  The problem is, I don't want to tell them.  I didn't want to tell them.  What's wrong with telling them everything is fine? They don't need to worry, they have a life to live.  I would rather hold all the pain and worry for them.  Ahh, the life of a mother who thinks she can save the world or at least her kids.

Spencer had some 'body work' done yesterday by a Rolfer.  A Rolfer does a deep, deep massage of the fascia which is the connective parts of the joints.  His Rolfer, Blaine is incredibly smart and has this 'sense' about people's body through the stress he feels in their joints and strangely enough, I knew him when my kids were tiny, I went to him for some Rolfins sessions but then he got cancer and I never saw him again. Well, he resurfaced in Spencer's life, long story and now Spencer hires him to come to the house and Rolf him.

About 4 months ago, Blaine told Spencer that he felt he had a major infection.  Spencer had just received some bad blood counts where his white cells were abnormally high and his monocytes were flagged high as well.  Spencer told him his counts were off and Blaine said, "Ya, I can feel that, your body is losing the fight just in case you were wondering."  We brushed it off and left it at that.  It's interesting that Spencer's bone marrow and the left shift confirms that Spencer is indeed losing a battle with an infection somewhere in his body.  Now we have to find it and we have a very short time to do this.  Blaine told Spencer a few more things yesterday which made Spencer question a lot of the bone marrow results.  Strangely, Blaine was right on about Spencer's body!! What a gift this guy has and funny he's back in our life.

We had a good talk and I came clean with Spence but not in an alarming way, still sort of sugar coating things.  It didn't really work.  Spencer is fearful that his mission will be put on hold or he won't even be able to go.  I assured him that he will go and he will be okay.  We have to have faith and we have to pray, pray fervently that things will work out.  Then Spencer said the thing I hate more than anything else in the world, "Well, I bet I know what happens.  I bet I will serve my mission then come home and my body will be so worn out, I will die."
"NO, no,no, it's not going to happen that way, things will be fine! This is just a hiccup, a test of our faith."

We can't afford to go there.  We have to stay in this moment no matter how uncomfortable this moment is.

Last night was emotionally hard.  I had to work really hard to keep from going to a place of worry and despair.  Shelbie is breaking down too.  She is worried about the boys and the fact that their marrow is much rougher shape than hers.  It's really hard!!! This week is just really hard.  I am trying with every fiber of energy I have left to keep everyone in a good place but it's hard.

Thankfully, I have no work to do this weekend so hopefully, Shelbie and I can find something distracting to do and we can both pretend to have fun with the big, grey elephant we are dancing around.  I know I will find myself honing my Faith and hanging out on my knees.  A little prayer goes a long way, even longer than Lindor Truffles!


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Thursday, June 13, 2013

Left Shift

Do you know what a left shift is?  It's not a step in the Hokey Pokey I'll tell you that much.

I got the written pathology reports from Seattle yesterday.  If you remember from a couple of posts ago, the nurse told me other than cellularity issues and the fact that Sam had normal telomeres , Shelbie had abnormal and Spencer wasn't tested, everything was fine.  ( I think that was just the world's biggest run on sentence hope you got all that.)

Well, I'm not sure what report that nurse was looking at but all is not fine!  Yes, the boys cellularity has dropped about 10% in a year.  Their marrow now appears to be functioning at just 20%.  Shelbie is at 30%.

Interestingly, Sam's telomere report is flagged as 'low'.  Low is not the same as 'normal' at least where I come from.  AND....imagine my surprise to see a telomere report on Spencer!!  They did do a telomere study and he too is flagged as 'low'.  Hmmmm, what in the world?!!

Now it starts getting really crazy...Spencer's report says that his marrow is 'Left Shifted'.  What that means is that there are more immature blood cells than mature cells. For some reason, often due to major infection, the body is being signaled to send out lots of new blood cells to fight but they never get a chance to mature either because of serious infection or bad cells like leukemia taking up the space meant for healthy cells.  This is a relatively serious condition and I am pretty bugged that the nurse didn't address this with me. It helps fill in the missing pieces since his white count has been abnormally high lately and his monocytes have been really high as well when we test his peripheral blood.  Abnormalities usually show up in the marrow first and it takes awhile for things to show in the peripheral blood but it's there.  sigh!

The other startling news is that Shelbie and Spencer had what is called Hemosiderosis.  It's an overload of a certain iron protein that is disease causing and usually caused from internal bleeding.  It is the left over protein from iron that the body is trying to destroy.  It's confusing but iron overload is not a good thing and can limit the opportunity for bone marrow transplant!

This has all come as a great surprise and now I'm left to sort all this out.  Very frustrating!!  Now, the name of the game is trying to keep my cool, be patient and hope that my faith will be strengthened as I prepare to let go of Spencer in just 6 weeks!  Life is hard, some days, harder than I imagined!


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Wednesday, June 12, 2013

What is up with Children's Hospitals?

Our last appointment with the Neurologist that I just ranted about, was in Riverton at Primary Children's Medical Center Satellite facility.

This was our first time back to Primary Children's since they did a bone marrow biopsy on Shelbie when she was just 5 years old and refused to sedate her.  They just strapped her to a table and did it and I'm pretty sure the local anesthetic hadn't kicked in before they started.  I also found out after the fact, that they had not sedated Spencer when he was 15 months old for an intestinal biopsy.  I only knew that because I asked for his records.  They noted that he was agitated and screaming during the procedure!  It's not right and after a series of errors and poor judgment and getting the 'Doctor of the Day' instead of our regular docs, I moved everything to St. Lukes in Boise.

We've been to Seattle Children's too.  There is a common thread between these two hospitals...they both employ the crankiest nurses and staff ever!  I will say that our last trip to Seattle Children's was slightly improved over previous trips.

Primary Children's was a joke this last time.  The only thing that resembled a cheerful countenance were their stupid Pooh Bear scrubs.  Other than that, we ended up with Nurses Cranky, Crotchety and Crabby! It doesn't help the stress levels and anxiety we all have at times like this when the staff can't even muster up a cheerful hello.

They were getting mad because Sam's blood pressure was 98/37.  I told them that that is what he always is. They said it was impossible and I was mistaken.  They took it again.   Again, it was the same.  They took it a third time, manually, the same.

They got to Spencer and the very same thing happened, a low blood pressure and heart rate.  They took it three times.  The nurse said, "You know that is not a good blood pressure don't you?"
"Yes, I do but I have told at least three doctors about it and no one has done anything to change it so what am I suppose to do about it?"
She just shook her head and basically ignored me, perturbed that I was not being a good mother or something, as if I could control my child's blood pressure.  Incidently, I mentioned again to the doctor that their blood pressure has been getting lower and lower over the past few months.  Nothing...crickets.

If I owned a children's hospital, I would run it like Walt Disney would.  There is no excuse for people in the healthcare industry to be grumpy and children's hospitals seem to be the worst at service with a smile.

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Monday, June 10, 2013

New Doc on the block

We saw our new genetics/neurology doc.  The jury is still out on whether I like her or not.  I have come to this conclusion though, geneticists are strange folks.  Maybe it's because they are so smart, they lack some social skills, I'm not sure but it was a weird and disheartening visit in many ways.

It's really hard starting with a new doctor.  You run the risk of not meshing from a personality standpoint but you also take a risk in the fact that they may not agree with the foundation that has already been laid and may  not mix well with the team.

All I really wanted was to address the latest neurological problems with the kids but of course, we had to hash up the entire past which took no less than 4.5 hours!!  It's was a long day people, a long day!  She was nice but methodical in the way she spoke but not in an effective way.  She had to say the same thing in about 5 different ways before she could really communicate her thought process.   I noticed that her physical exam was different on each kid which made me think that she really missed out on some tell-tale problems like Shelbie's extremely sensitive reflexes.

I kind of laughed when she put gloves on to touch the kids' feet.  She said, "Sorry about the gloves but I can't stand feet and I don't like touching them."  I thought that was very strange for a doctor to say.  A doctor...interesting.

Anyways, I was doing okay with it all until the very last few minutes when she decided to tell me about her philosophy and what she thought about the kids. She doesn't believe in labels.  If there is no concrete diagnosis then the kids' chart lists nothing.  So, according to her, my kids don't have Shwachman Diamond Syndrome or Mitochondrial disease.

I questioned her and said, "Well, can we agree that my kids have bone marrow failure, mitochondrial and neurological problems?"
" They do but that doesn't mean they have mitochondrial disease or Shwachman Diamond Syndrome.  I think labeling people with a disease does them a dis-service because anything else that goes wrong, they try to fit it into the parameters of the label.  Not only that, it scares patients to tell them they have mitochondrial disease since most patients die and there is no treatment."

At this point, Shelbie starts crying and boys are about to jump out the 3rd floor window.  I had to come to some common ground before we left.  I can appreciate to some extent what she said but it's pretty hard being a chronically ill person and having no idea what is happening to you.  From my kids' standpoint, they would rather have a name for their problems than to be told they have nothing that has ever been described.   Welcome to the world of orphan diseases!  It's much easier to handle news, especially bad news when you have some context or framework to put it in.  Without a doubt, we are in the right sandbox with the mito and SDS diagnosis whether she wants to admit it or not and just because they haven't found the gene doesn't mean they don't have it.  It's so frustrating.

We finished the day with her saying, "Of course you are in a very uncomfortable place and without knowing what your children have we also don't know how to treat them or what the course of their life will be and that is hard no doubt.  I know I can find a diagnosis, you will just have to be patient."

At that point I just had to get out of there.  What I really wanted to say was, "Doc, we have been working with the top doctors in many fields, all trying to find the defective genes in my kids for 21 years!  What makes you think you are going to find the answer anytime soon?"  I'm not being patient and she has no idea how hard this is.  We left with no help for the current problems which is exactly what happened when we left the Neurologist last June.  They stare at the problems, scratch their head and say goodbye and tell me to just 'watch' them oh, and they take another 10-12 tubes of blood which I will probably never get results on or even know what they ultimately do with all that blood.

Seriously, at one point, she was checking Spencer's gag reflex after I told her it was gone.  She didn't think that was likely so she checked it.  She had her entire fist in his mouth and not one but two fingers down his throat...nothing.  Not a cough, not a gag, nothing.  Did she acknowledge that that was not normal?  Nope, she doesn't want to call it something and run the risk of scaring Spencer.  Well, too late, Spencer lives everyday wondering what is happening to him.

I'm just about done ranting...sometimes, I feel like I live in a parallel universe out in the middle of a twilight zone/black hole.  Is there no one who can help us?  Arghhhh

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Friday, June 7, 2013

Dumb and Dumber

I've been feeling pretty dumb lately.  Just tired I guess and nothing seems to make sense and we have more questions than answers but then, the phone rings.  It's Seattle.  Bone marrow biopsy results are back.  "We have good news and bad news." the nurse says.  She didn't give me the option of which news I wanted first, as is customary, she scrambled it all up in one big serving of delightful confusion!  

It's not very often you hang up the phone with a several less brain cells than when you cheerfully answered the call! Yes, I was dumber when I said goodbye than when I said hello!

Here is the brief synopsis.  I'm sure I will have more to say when I get the written report from pathology.  The boys did not have stable marrow.  Their cellularity was considerably lower than last year's biopsy.  They have to start having more frequent blood counts done and if those don't improve, then we have to have another biopsy in 6 months!  Stinks.  

Shelbie's marrow however, was stable from last year.  Yippee.  The bad news on Shelbie is that the doctor is even more convinced that Shelbie has Dyskeratosis Congenita because of her abnormally short telomeres and an increase in symptoms but, she doesn't want to test for that quite yet which I totally don't understand but whatever!  They just got consent from our insurance company to go ahead with the B800 Genetic testing.  Don't even ask me what that is...I didn't even know about that testing until the phone call.  FYI- Dyskeratosis Congenita is even more rare than Shwachmans.  Last I heard, there were 59 children in the United States with DC...59!! That's not very many.  Lucky for us, one of the experts in that field is our great doc in Seattle who is also an expert in SDS.  So, that's great. 

Now, here's where my mind blew up.  This time, they tested Samuel's telomeres but not Spencer's.  Guess what?  Sam has normal telomeres!!!  What the heck?  Could that mean that these kids of mine were all born with something different?  Is that even possible?  How could that be, seriously!!  I have never felt so ill equipped to handle a situation as I have this one, in this moment!  Blows my mind. 

The thought crossed my mind that there is no way Spencer is going to be able to have his blood drawn every month while he is on his mission let alone having a bone marrow biopsy in 6 months if that is the way things go.  I don't know a soul in Colorado from a medical standpoint.  I'm sure our local docs would help facilitate that but it adds a freakish dimension to letting him leave.  I just have to put it out of my mind and have faith that it will all work out!  I kind of had a feeling that Spencer would go down hill just before he leaves.  I think God is just testing me to see how much faith I have.  So, that's fine...onward and upward.  

Wednesday was the longest day in all of creation.  Thursday, we left at 5 am to drive to Salt Lake where the kids spent the better part of the day at Primary Children's Medical Center Riverton, with our new Neurologist...It was not fun but more on that later. 




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Tuesday, June 4, 2013

A first...

Tonight, we experienced our very first Asthma attack.  I'm talking full blown, can't breathe, loss of vision, dizzy, asthma attack.

We've had near attack moments but nothing like this one.  Spencer had been at the gym tonight, just like he is every single night.  He was with a friend and they decided to jog home.  I would say the gym is only about 5 blocks, maybe a bit more but not miles by any means.

Spencer flew in the front door and stumbled into the kitchen!  His friend was close behind.  I was thinking they were just goofing off and made some wise crack like, 'Hey mister fitness gasping for air, can't handle the fun?"  Something stupid anyways until I realize he is scrambling to get his rescue inhaler and then I felt really stupid.  He was so short on oxygen, he had lost part of his vision halfway home.  The room was spinning and he could not get air moving.

You hear of people dying from asthma and I have never really been able to understand that but I think tonight, I get it.  At least a little more than I did.  It's strange though because Spencer really is extremely fit, I mean if you can classify a kid with bone marrow failure and mitochondrial disease as fit.  He's got some serious muscles on that tiny frame. You wouldn't think 5 blocks would do him in nor would you think that after living with asthma for 15 years, we would have experienced an attack sooner than this.  I guess we've been doing a good job of managing things but this came from out of the clear blue.

Sadly, I wasn't a very effective nurse.  Outside of the rescue inhaler, I really had no idea what to do for him and I didn't know at what point you have to obtain emergency care.  I guess I have some homework to do.  We both do.  I think that maybe this was a wake up call that he needs to carry his rescue meds with him at all times, especially when he leaves on his mission.  He will be biking up steep mountain roads and needs to know how to take care of himself.  So, even though it was a tad scary, it was a blessing in disguise.  We will be a little better prepared now.

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Saturday, June 1, 2013

Watch Out Disney...And Give Kids the World

This is where we will be staying- Give Kids The World


As I mentioned in my last post, Sam has been granted a wish from Make A Wish!  His wish was to go to the biggest, scariest, fastest, baddest roller coasters in the United States!  He had three places in mind, Disney, Cedarpoint in Ohio and Six Flags in California.  He did a lot of research on which roller coasters are in which park.  I think Cedarpoint was his first choice but Disney had a little more to offer so he went with Disney World as his first choice.

So, Disney it is.  He doesn't know he is getting a wish yet.  He has been hoping that he can go before Spencer leaves so he has some company on the roller coasters.  I hate roller coasters but I will ride them for Sam.  You watch, I will!

He is still a little traumatized from the Hurricane Sandy in New York last fall.  He brings it up a lot and asks a lot of questions like, "What kind of natural disasters does Florida get if I go there?"

"Hurricanes." I say
"Whhhhaaaatttt?  Oh man, not another hurricane!  Do they get earthquakes or tornadoes?"
"No, not tornadoes, Ohio does though.  Sam, we are old pros at hurricanes now.  Been there, done that!"
"Oh geez, maybe I should just do a shopping spree at Walmart or something cause you know something freaky is going to happen to us!  With my luck, I will be at the top of a roller coaster and a tornado will come out of nowhere and suck me up and I will never be found."

It's funny, but it's not.  It's somewhat true, we have the weirdest luck!  We can't even go to McDonald's without experiencing something strange.  Even their dad commented on how weird things get for us.  I guess it's me.  I hate boring and normal!

I'm trying to figure out a fun way to tell him.  I was thinking it would be fun to surprise him just an hour before we leave.  Have his suitcase packed with brand new shorts, shirts, sandals and sunscreen.  Balloons, Mickey Mouse ears, you know the hats, not his real ears...duh!  Just kidding.  Maybe a little more Disney stuff.  I think he will be shocked because we leave just two weeks before Spencer leaves so he is going to assume it won't work out.  Of course, I will play up the fact that it's never going to happen.   I get excited for him just thinking about the surprise!

Feel free to respond with any ideas...and if you have any Disney tips.  Shelbie's been doing some great research on all the freebies and deals available.  We will be staying at the coolest place on earth, Give Kids The World.  Ice Cream Sundaes anytime of the day, it's like it's own little amusement park.  Here are some more pics of our accommodations.  It's a resort in itself and just for kids with life threatening illnesses.  Keep in mind, the following pictures are just our 'Hotel'.





I can't even believe the fun that Sam is going to have!  Make a Wish has been a blessing for the kids!
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