Monday, January 31, 2011

Hard Decisions

Shelbie's platelets are below 10 and today she is really feeling sick.  Definitely worse than yesterday or Saturday.  I finally got our doctor to schedule an infusion for tomorrow morning but he sent me an email saying that he is not comfortable doing more infusions and wants to meet with us to discuss a new treatment for ITP called Thrombopoetin Agents.
      I already know that our Hematologist in Seattle is very much against the use of these new drugs in my kids.  One of the major side effects is that is causes fibrosis of the bone marrow or in other words, scar tissue.  If they build up scar tissue, their chances of surviving a bone marrow transplant or a transplant with few complications becomes slimmer than they already are.  Not only that, the drug can decrease cell production in the bone marrow and can increase abnormal cancer causing cells.  Well, all three of the kids have some abnormal cells in their marrow that can develop into cancer on their own, let alone with the help of some drug.  The kids are sitting at about 20% cellularity now, they can't afford to have more of a decrease in their cells.
       Further more, the pharmaceutical company warns that these new drugs are not meant to put the disease into remission or even raise the platelets to a normal level, only to get the platelets to a point where internal bleeding becomes less of a risk.   Finally, the new drug can affect the eyes and cause vision changes.  In light of the recent DNA mutations that could potentially cause the kids to lose their vision, I am not happy about the risks involved with this drug. 
      In a nut shell, my mother senses are tingling and I think changing to this new treatment is a very bad idea.  The current treatment of IVIG is not great because of the headache and nausea that it causes but it too brings the platelets to a level high enough to eliminate the worry of internal bleeding.   There are no other risks involved with the cellularity of the bone marrow etc.  It's pretty safe as far as long term treatment is concerned.  There are lots of SDS kids who have been getting monthly infusions for years and have had no adverse affects. 
      It's going to be really hard to sit down with our Oncologist and stick to my guns of what I feel is best for my daughter.  It will be his scientific mind against my emotional, mother bear mind although I have done my fair share of research so I don't think I am being entirely irrational.  I sent off an email to Seattle in hopes that our Doc there can at least give me some feedback and reassure me that I am not being irresponsible by declining to try this new medication. 
       Ahhhhh, these are hard decisions!  I don't like making them.  I wish God could send me an email or telegram with the right answer written on it.  All I know is, I look at Shelbie and my heart just breaks.  I  like to think I know what she is going through and how hard it is but I really have no idea.  I know she feels crummy and anxious but it's still hard to be the one that she takes all her frustrations out on.

Sunday, January 30, 2011

Make A Wish- 2007

These are never before seen pictures of Shelbie's Wish in 2007.  We surprised her as she came out of her voice teacher's house.  She knew she was getting a wish but she didn't know when she would get it or if they would be able to grant her 1st wish.  Her 1st wish was to record a vocal CD and take it around to sick children in the hospital.  Her 2nd wish was Disney World.  I remember her saying, "Disney would be fun and I would be happy with it but I really hope I get my first wish."  She did!
The boys made her this fun sign.

All of us waiting for her to come out of the house.  Photographers from the Post Register showed up and actually did a front page story on Shelbie and her wish.

Does this guy look familiar?  I wasn't kidding when I said in my Christmas post that he was an important part of our family!  He was the guy to give Shelbie the great news about her wish along with two 'Wish Granters' from Make A Wish!

Surprises are so fun!

A look of disbelief!

I just love this picture

Coming to say hi to her wish granters and family

The wish took place in Boise.  On the first night, we attended a Make A Wish banquet/fundraiser.  Each table is assigned a team from Boise State.  They assigned Shelbie the Football team!  They waited on us hand and foot and were so kind to Shelbie and the boys.

More of the football team

The Quarterback teaching Shelbie some dance moves.  They shared a favorite song and got up on stage later in the evening and danced together.  It was so much fun!

Waiting in the hotel Lobby the next morning for our limo to arrive.  Shelbie was pretty good at covering up the bald patches on top of her head with this fancy comb over.

Inside the limo.  A flat screen tv and on the other side was a bar stocked with all sorts of family friendly drinks!

Sound check at the recording studio.  She recorded 7 songs in 10 hours.  A super long day but well worth it!

Getting ready to record the first song.

When we got back to the hotel room, the Wish people had decorated it all up with records, music notes and twinkling lights along with a huge basket full of candy!


The following two days, Make A Wish kept us busy with various activities.  The second to last day of our trip, they presented her with 300 finished CD's a trip to St. Lukes to visit the kids on the Oncology  floor and other areas of the hospital.

Back cover of CD

The CD

Signing Autographs for the kids. 

She ended up distributing all 300 CD's with exception of a few we kept for our family.  When we got home, she had 'appearances' to do at Macy's, Z103,Classy 97 and Q102- local radio stations.  They played a selection of her songs and so many people called in to talk to her after they heard her songs.  It was amazing to me how many lives she touched with her music. 
        We will be applying for the boys to get a wish this year.  Make A Wish calls me several times a year to see how Shelbie is doing and if they can grant the boys a wish.  I keep waiting until just the right time...I think that time is coming soon.

Friday, January 28, 2011

I remember why...

If you have been following my other blog, you will notice on the 'Archive' list that I didn't blog for all of 2007.  You would think that year didn't even happen if you go by the blog.  Well, it happened alright and tonight, I am remembering why I didn't bother to blog all year long.
     2010/2011 feels like a rerun of 2007!  January to February of 2007 were two months of really sick kids.  By March, Shelbie ended up with low platelets and that nightmare continued until December.  The only difference in 2007 was that she ended up having chemotherapy treatments every week for nearly 6 months!  Chemo was by far the worst experience we have endured. Someday, I'll have to post pictures of my Shelbie Girl with only half a head of hair!  (when I have my computer running again and have restored my hard drive, I will post some.)  Not only that, she was so sick!  I remember the very first treatment.  I have never seen anyone so sick in my entire life.  She was laying on the bathroom floor sobbing in between vomiting and in the middle of that, the door bell rang.  I ran to get it and it was a good friend of mine from my book club.  I was sobbing, shaking and fell upon her shoulder and begged her to make it all stop.  It was a pitiful moment for us all.  She came to my rescue that afternoon and helped me get Shelbie cleaned up and off the floor. 
       Back then, I remember clearly thinking that I did not want to remember any of that year.  It was so painful to live through as a mom.  That's why 2007 went unrecorded.  We went through all of that while I held down a 50 hour a week job and homeschooled Shelbie and tried to keep the boys sane. 
     I don't know how we are managing to get through this again!  I don't have a job which is probably a blessing but it still seems just as hard as it did 4 years ago.  I feel like I am starting to sound like a broken record.  "Platelets have dropped...", "We are tired, scared, frustrated....blah, blah, blah...."  The thought has crossed my mind that maybe I should consider pretending this year doesn't exist either!
     The truth is, this is real, this IS our life.  It's not easy, it's not fun...sometimes!  Other times, we have a blast in spite of the troubles and trials!  I guess that's why it was so important that I keep two separate blogs, keep this side of life in it's own little compartment so it doesn't overtake the rest of our existence.  SDS has already taken too much from me, I refuse to let it own all of me and what is important to me!  You may even notice that I can post the horrible and dreadful things going on and minutes later, report on something silly, fun, pointless or stupid on the other blog.  Life is still good no matter what the emotion of the moment. 
    Tonight, is a broken record sort of post.  Shelbie is so upset tonight.  With her platelets dropping by thousands each day, I can only imagine that tonight, she is well below 10.  All day, she has felt out of sorts, bad headache, extreme fatigue, achiness and is now covered in petichae; broken blood vessels.  She is so tired but admitted that she hasn't felt like this before so what if tonight is the night she goes to bed and doesn't wake up?  I have done all I can to reassure her that lots of people suffer with this disease and don't just die in their sleep.  Easy for me to say!  I feel so bad for her, so helpless.   I promised that I would stay up through the night to keep checking in on her so she could rest.  She has a photo shoot tomorrow afternoon and really needs some sleep so she can work.  I probably won't make it awake all night.  All nighters aren't as easy as they use to be!  I can't steer this river!  Even I know there is nothing about this disease that I can control, all I can really do is leave it in God's hands!  Shelbie knows this too.  She even said to me, "Well, if it's my time to die then it's my time and whether you stay up or not won't change anything."  Ughhh...do you know what it's like to hear those words come out of your child's mouth?  I so much admire her faith, courage, strength...I couldn't be that brave!  I feel like she will be okay but this disease sure takes an emotional toll that's for sure and it takes work to not let it overrun every thought.

Thursday, January 27, 2011

Getting use to life

Platelets are still dropping, surely and steadily.  It's so frustrating to see your daughter's blood in such a shambles and there is absolutely nothing you are anyone else can do about it!   This afternoon they are at 24 but there is still no plan to do an infusion this week.  I am torn between being patient with the doctors and letting them do their job and being a mom and listening to my daughter who is scared, tired and worn right out both physically and emotionally.  There is something unnerving about living with this disease and knowing that at any moment, without any notice, the cell walls can just collapse and internal bleeding will ensue.  There is no magic platelet count when this event could even be predicted and that makes it all the more annoying. 
     Sometimes, I think that a person's emotional well being is just as important as their physical.  Would it be that big of a problem to do the infusion sooner than later just to ensure a little more peace of mind?  Apparently, yes, that is too big of a problem and too much to ask.  Whenever we have had setbacks in the past, we have adjusted fairly quickly and established new patterns of normality.  Not this time though.  I'm not sure what is different but I'm just not sure we will ever get use to this part of life, the up and down, unpredictable platelet counts.  Maybe there has just been too much going on this year and life has taken it's toll.  Who knows.  We will just have to keep hanging on, sometimes by the very tippy, tips of our fingers.
       

Wednesday, January 26, 2011

St. Lukes

View from the hotel looking down on the river.

St. Lukes- Boise

Shelbie

All Spencer wanted was an A&W rootbeer in a "Frosty Mug"  He finally enjoyed one on the way home in Rupert

Made it home from Boise tonight.  The weather there, though foggy was warm, warmer than here at least!   You'll notice from the pictures, there as not a spec of snow, it was so nice to be sure of your footing instead of dodging the ice patches.
I love St. Lukes.  It is by far my favorite hospital ever.  Primary Childrens doesn't hold a candle to St. Lukes.  We had a great visit with the Gastroenterologist.  It was nice to have face time with him instead of just emails.  The kids are still small, of course but to put it in perspective, Spencer just reached the growth charts!  It has only taken him 16 years to make it onto the charts and now sits proudly at the 2nd  percentile for height and weight!  He has gained nearly 6 pounds in 13 months.  Sam wasn't quite as lucky, in fact a little disappointed to hear that he only gained 1.5 pounds in a year and grew 2 inches.  He sits on the charts at around the 4th percentile.  Shelbie is holding steady at 4'10", a little bit of a girl. 
It took a couple of days to get the tests completed so we will have to wait a week or so for the results.  I am not expecting anything too earth shattering.  I do expect that they will be in bad shape as far as vitamin stores go and I'm sure they will have to hit the Vitamins A, D, E and K pretty hard.  Shelbie couldn't have some of the tests done because her platelets are so low so we will have to wait on those. 
It is really hard to figure out what is going on with the boys and their constant nausea.  He suspects that since they have both had major surgeries in the last year or two things have changed in the way they move food through.  Sam especially with his bowel surgery is messed up!  The doctor is starting both boys on new meds so we will see how they do on those.  In three weeks, if things are not significantly better, we will have to look for new treatments to help them.  As I type this, both boys are upstairs with their favorite puking bucket, not feeling so well. Sigh....  This brings our medication count up to 38 pills and concoctions to drink per day between all three kids.  I decided on the way home that I definitely need to come up with a better way of distributing medication so it's not so time consuming. 
That's all I have to report on for now.  Tomorrow, Shelbie will get another CBC and the remainder of the week will play itself out.  Infusion maybe on Friday?   hmmmm, we'll have to see!

Monday, January 24, 2011

Adventurous Day

There is just never a dull moment in our little lives.  We left for Boise this afternoon hoping to stay ahead of the snow that was forecasted for the area.  We stopped just before Twin Falls at a truck stop called the Garden of Eden.  I went into the bathroom and laying on the floor was this elderly woman.  There was another woman kneeling down beside calling out for help.  I ran over to her and she explained that her mom was having a stroke.  I really didn't know what to do.  The lady kneeling beside her was the daughter and she wanted me to help stand her up.  I got my arms under hers and tried to lift her up but there was no way this woman was going to be standing.  She was totally out of it.  Her heart seemed okay and she was breathing on her own but completely limp and unaware of what was happening.  I laid her back down on the floor and ran out to the service counter to call 911.  Thankfully, there was a nurse at the counter paying for her gas purchase and came in to help us out.  While the nurse was attending to her, I found the husbands of these women and got some blankets from the car to keep her warm while we waited for the ambulance to arrive. 
     The woman was 87 and her husband was 94 and they were driving with their daughter and son-in-law to Palm Springs.  This was her third stroke in the past few months.  We ended up staying at the truck stop for just over an hour until the woman was okay and we got our blankets back.  It felt good to be able to help even though there was nothing more I could have done.  It was so sweet to see this woman's husband kneeling beside her trying to wake her up.  He held her hand tight.  When she started becoming more aware of her bad situation, her husband gently touched her cheek, "Honey, what are you doing down here?  You will be okay."  It was so touching. 
      While the paramedics were in working on her, a tour bus of elderly folks from Alberta pulled up and all these people crowded in to use the bathroom so we then had to direct all those people to a bathroom down in the basement since the main bathroom was full of medical staff and a stretcher.  The gas station staff was happy we were there to help. 
      Anyways, we got to Boise safely, not a spec of snow here and tomorrow it is suppose to be above 40 degrees!  Awesome! 
      I spoke to the doctor this morning about the platelet problem but they are hesitant to treat her right now since it is so soon after the last one.  This is kind of a hard place for Shelbie to be and I worry too.  We will just have to hope she doesn't a worse bleed than she has already had and that she will be able to hang in there for another week or two.  Dr. H assured me that coming to Boise would be okay.  He said they have had women deliver babies with a platelet level of 60!  Scary if you ask me.  It's still unclear why the infusion didn't work this time.  We have to do what we have always done, be patient.  There are no answers now, all we can do is live the questions and wait for the purposes of this to unfold. 

Sunday, January 23, 2011

The week ahead

I am quite certain this week will prove to be a busy one.  I was perfectly content spending the weekend in a state of distraction.  There is however, only so much you can do until you have to face reality.  I ended up taking Shelbie up to the hospital for a CBC this afternoon. 
     I had this random thought that since we have been trying to do fewer blood tests in an effort to preserve her veins we didn't check any blood work since her infusion.  This means that maybe the platelet count on Friday was actually showing that they were on their way UP not DOWN like I had assumed.  It's just that we are use to seeing a sharp incline right after IVIG so I figured this time wouldn't be any different. 
     The Phlebotomist we had today was so nice and had a great sense of humor.  That is always helpful at times like these.  He said, "Okay, are you ready?  This won't hurt me a bit.  You close your eyes and I'll close mine!"  I thought those were all clever lines plus he was pretty darn cute...oh wait, it just occurred to me that maybe he was flirting with my daughter....okay, not so cool!
     So, anyhoo, her platelets have dropped 20,000 and she is at 45, also not cool.  I have already emailed the doctor so he will see the results first thing in the morning.  I am anxious to hear what he thinks about this.  I pray that she has not developed an immunity to this treatment already!  Oh how I'm praying for that.  I don't know what other options we have left that would be safe now and not cause any problems for any future treatments.  There has to be something else contributing to the platelet destruction besides the IgG levels but what? (sigh)  Going to Boise remains to be seen.  I will feel so embarrassed if I have to cancel our GI doc again!  He pretty much blocks out half his day just for face time, the rest of the visit will be spent doing testing, so us cancelling leaves him with a big chunk of time and lost revenue.  Sadly enough, I may not have an option in this.  Tomorrow will be a big day, one way or another.  I can hardly wait.  I'm sure the excitement of it all will keep me awake and giddy all night long!

Friday, January 21, 2011

Hmmmm...There goes that theory

Well, I got home from the temple this morning and Shelbie was sitting on the couch waiting patiently, all dressed and ready for the day.  Somethings up when Shelbie is dressed and ready to go so early in the day.  She told me she needed to get a blood check since she had had a big bleeding episode this morning when she brushed her teeth. 
     I figured it was just a fluke thing, it's only been 11 days since her last infusion and the IVIG quantitative tests came back high which should mean that there shouldn't be platelet destruction this soon in the process.  We headed up to the hospital and got a CBC done.  We sat at waited for the results and played our usual game of 'Guess the platelet count'.  I guessed 125, It's always 125 on week two post IVIG.  Shelbie guessed 95.  We both lost....her platelets were all the way down to 65! 
     This leaves us in yet another quandry....I give up!  I rescheduled Boise for next week because this happened to us last time.  Now it is happening again.  The doctor doesn't work on Fridays which means we won't get instructions until Monday but we are suppose to leave on Monday for St. Lukes.   I am tempted to go anyways, heck, we will be in a hospital.  We will be a 2 min walk away from our old Oncologist at the Mountain States Tumor Institute.  I'm know they would welcome us back and help Shelbie if she gets into trouble with her blood. 
    Isn't life interesting?  Sometimes, when I am not freaking out, I can stand back and just see the spread of our life and how everything plays out.  It's always for the best, but can be hard to see when you are in the moment.  Maybe it's good I am so tired, cause for some odd reason, this doesn't even phase me. Makes me wonder though what will happen next.  According to the recent studies, she has so much IVIG in her system, she really shouldn't be experiencing platelet destruction.  So much for that theory! 

Thursday, January 20, 2011

Waiting...

Waiting....at least we are use to waiting.  We got the results back yesterday that both doctors were waiting for.  One was her IVIG levels now that she has had three months of infusions and the other was an Epstein Barr Virus titer.  They tested her for Epstein Barr which is basically Mono because she has these swollen lymph nodes in her neck and runs a low grade fever about every other week.  The EBV titer came back normal which is good but doesn't solve the mystery that is causing her lymph nodes to be large and the unexplained fevers. 
    Dr. S in Seattle was concerned of some bigger problems brewing so she will be conferencing with our Onocologist here to decide on a game plan.  She had mentioned to me that if it was negative, she would want to see Shelbie in her clinic within the month.  I am not sure when the decision will be made but a road trip might be in our near future.
     The IVIG was high, 2000 which is good.  It means that she has plenty of arsenal to fight the antibodies killing off her platelets.  With it this high, there is a good chance she won't need an infusion next week, maybe not even the week after.  She may actually get a little break...yay for breaks! 
    In the meantime, I hope to catch up on some sanity this weekend, maybe a little sleep and prepare for Boise next week.

Wednesday, January 19, 2011

What's in a name?

There's a lot in a name.  Part of the frustration is feeling like a science experiment.  Each kid seems to come up with new problems all the time and their list of diseases is growing.  It's hard for me to wrap my mind around the fact that they have so many problems that seem to get treated individually.  How does one person have 4-5 different diseases?  There has to be one name that sums up everything.  It's not often that people understand this. 
     Yesterday, I expressed some of my anxiety over that and our doctor actually agreed that not having a name is very difficult and not just for us.  Without a confirmed name for this disease, she can only guess at the right treatment.  We have already experienced firsthand what happens when they treat the kids under the assumption that they are 'normal' kids with a few strange problems. 
     In 2007, Shelbie had this same problem with her platelets.  After many failed treatments, they turned to their last resort, VinCristine, a chemotherapy drug. In most ITP patients, Vin Cristine would work wonderfully and with very few side effects.  For Shelbie, she was so sick.  Sick to the extremes and her hair fell out, something they promised her would not happen.  As it turns out, we recently learned, on one of our Seattle trips that SDS kids who get VinCristine will have extreme side effects and it can actually harm their marrow and cause irreversible damage. 
     It's important that those kinds of mistakes don't happen again!  I guess that's why Dr. S had a bit of worry in her own voice and I sense she really is in a hurry to call this disease by it's appropriate name.  There is some discrepancy about how long Shelbie should stay on IVIG.  Our local Oncologist wants to end those treatments in April and begin a new drug.  Our Hematologist in Seattle is very much against the new drug because it is known to cause scar tissue to build up in the marrow.  Scar tissue in the marrow makes a transplant very difficult and risky.  I am confident that the two doctors will come to some common ground but when you start weighing out the risks that come without knowing the exact name of this disease and the process by which it destroys the body it is really scary. 
     Before Dr. S and I finished our conversation yesterday, I asked her what I should call the disease my kids have.  I just need something to hang my hat on .  She said, "They definitely have Bone Marrow Failure Syndrome, and I am comfortable calling Sam 'Clinically SDS' and Shelbie and Spencer are definitely in the arena of SDS.  For now, I am treating them as I do all my SDS patients and refer to them as SDS kids, this is what fits them the best."  I guess for now, that will have to do for me too. 

Tuesday, January 18, 2011

Mitochondrial Results

Well, I got the call today from Dr. S in Seattle.  She started out with, "I have some good news, the kids do not have mitochondrial disease affecting their marrow!"  Inside I'm cheering, squealing, relieving (I know that's not an adjective but today it is!)  I try to come across as calm, "Great, that's great!"  She continues, "but..."  There's always a BUT!  I hate BUTS. 
"They do have a surprising sequencing mutation that causes a mitochondrial disease called Lebers Hereditary Optic Neuropathy but we aren't sure what that means for your kids or what to do with this information." 
      A sinking feeling takes over, how can this be?  Eye disease on top of every other stinkin thing?  From there, the conversation was overwhelming.  When she saw these results, she met with the mito docs to see what they thought.  They too were stumped but wanted to see the kids in June when we go for bone marrow biopsies.  Dr. S also contacted one of the world's top Biochemical Geneticists, the best in the world!  She is Dr. K and incidently works out of a clinic in Seattle.  She mostly only studies large families with completely unknown and extremely rare and severe diseases.  She studies their genes and tries to discover which genes and chromosones are mutated; a huge job.  After Dr. S met with this famous Dr. K and they discussed my kids, Dr. K decided that the she wanted to take us on as a project, free of charge!  This is an amazing blessing.  Clearly, God had a hand in this.  Why else would she give us the time of day?  She is confident that her team will be able to get closer to what mutated genes are causing all these problems. She even said there is a big possibility that they will discover a new disease within the realm of SDS!   Even though this feels good, it is so overwhelming and terrifying.  I don't even think I can explain how devastating this feels tonight. 
      We also discussed what has been going on with Shelbie and that is not looking good either.  I will probably save those details for the next post, I am wiped out tonight!  The short of it is that she will no longer be getting a port as planned and we may be making a trip to Seattle in the next few weeks instead of June.  Stay tuned for more details.

Sunday, January 16, 2011

Melting Down

I guess when you are going through the refiners fire, you're gonna have moments of complete meltdown.  This weekend has been filled with melt downs, temper tantrums, and heart breaking drama.  I guess the events of the last two years but more importantly the last 6 months have begun to take their toll. 
      I don't know what my problem is but I have not been in the game this week.  Truth be told, I have been a horrible mother.  The kids have been sick and normally, I am helpful and dote on them until they feel better.  This week, I just didn't have it in me.  I was sad that they didn't feel well, but it's to the point that there is nothing I can do to change things.  I felt so helpless and that made me feel angry.  By Thursday, I was so done with the week.  Shelbie and I had all sorts of run ins during the week.  She was miserable and expected me to be more sensitive than I was.  It's my fault really, and I felt so bad.  I told her I would be better for the weekend and we tried to plan something fun for Saturday. 
       She went to get her hair done and then we had planned to go shopping in town.  Well, her hair took forever, almost 5 hours so she was tired, sick and hungry but we went anyways to find her some dresses.  The rest of the afternoon went downhill from there and we ended up fighting again!  The whole weekend was just a big waste!  Saturday night, we both ended up in tears and both of us finally admitted that life is just too hard right now.  We are both dealing with a lot but doing our best to keep that stiff upper lip and pretend that everything is OK.  Well, all the 'ok' finally burst out in emotion that has been hurtful and discouraging. 
       It felt good to finally melt down. Nothing was solved of course but sometimes a lot of tears is a good thing.  I suppose there is no sense in trying to steer the river.  There are no answers, only a bunch of restless questions.  It's tough but it is what it is.  I really hope I can pull it together this week.  I have had enough of being tired and overwhelmed!

Thursday, January 13, 2011

Headaches...

Headaches must be the theme for this week.  Aseptic Meningitis finally arrived at about 9:30 on Tuesday night.  I really wanted to avoid the hospital and we did but it was rough and maybe not worth it.  The worst part was that we didn't have any pain meds for Shelbie so I was trying to treat it with huge doses of Tylenol, Advil, Celebrex and finally just gave her something that I hoped would help her sleep it off.  She was up about every hour and half with so much nausea and pain, it was a really long night. 
      Usually the following day, the symptoms are manageable but not this time.  They stayed pretty bad all day Wednesday and into the evening.  She finally went to bed about 8:30 because nothing was getting better and I had no relief to offer her.  Today, she is almost back to normal.  Super tired but she was able to get up and go to her tutoring job for an hour or so. 
      I titled this post Headaches because Spencer came home really sick from school today.   He can hardly bare the pain from his headache that starts at the base of his neck.  He also has a sore throat, low grade fever and body aches but  my first thought was seizures and encephalitis since this kind of headache he described happened about 8 years ago when he got encephalitis.  That was a miserable month long ordeal.  I guess I tend to live in this hyper alert state all the time now, I never believe it's just going to be something simple anymore.  Only time will tell what Spencer's illness becomes.  I hope it isn't too bad like strep or influenza.  Suppressed immune systems and nasty viruses and bacterias are not a good combination.  Here's hoping for the best...or at least to survive whatever is waiting around the corner!

Tuesday, January 11, 2011

A little light

There was a glimmer of light in the day when I was able to meet with our Oncologist.  I had forgotten that I had scheduled the kids' quarterly check up for today.  I noticed it on my calendar just 10 min before the appointment so I rushed over to his office.  I decided to leave the kids at home and just have some time alone with him. 
    It was a good visit.  We talked about the lack of communication, a care plan, a prognosis and all those things that have been on my mind and causing worry.  He acknowledged that his staff knows all about leukemia but nothing about SDS.  He said almost word for word but I said over a month ago so it felt good that he understood the isolation we feel.  He gave me all his contact information so now, we will just bypass his staff.  I really appreciated that.  He also feels that it is pointless to have 5 different specialists all working on their own thing instead of everyone interfacing with each other but to make that happen will be next to impossible.  How do you get everyone on the same page?  I'm not sure how to accomplish that but at least I'm not the only one thinking it is crazy. 
     As for a plan...We don't have one!  Seriously, we really don't.  I did learn a few things I didn't know about IVIG.  Because it is a blood product that comes from so many different people, over time, it can just stop working because the body begins building up antibodies against all 'donors'.  At least this is how I understood him.  So, the more infusions she has to have, the higher the risk that it will just stop working, then we are left with a smaller arsenal of things to sustain her.  I don't want that to happen, we will need this treatment down the road as her marrow becomes more depleted and sick.  We decided that we will try to stretch the time between infusions to 4 weeks instead of 3, even if her platelets are low.  However, if they are at 80 but she is having nose bleeds or bleeding gums, then she will need to be infused sooner.  If they are at 30 at week three but there is no bleeding, we will try to hang in there until week 4 when she will receive the infusion. 
     I asked him how long he felt she would be suffering with this.  Of course he isn't God so he really couldn't predict.  We hope that her body will go into spontaneous remission but realistically, it could go on most of the year or never go away.  We've already been at it nearly 3 months!  He does want her to get a Port a Cath so that will be done next Thursday when her counts will be above 100, hopefully.  It will be an outpatient procedure and done at the local radiology clinic. 
     We are seriously going through some big time growing pains as we try to find a new normal and cope with this past year and the advancements of the disease that I was not prepared for.  The kids were not prepared for.   I would say we haven't been coping very well lately but I'm not really sure how to combat that.  Maybe time is the only thing that can make this better.  I am so glad that I was able to visit with our Doctor, it made all the difference in the world just to be understood even though he couldn't fix anything.

Monday, January 10, 2011

Salvaging the night

Whew!  Finally got home.  There were only a few tears tonight as Sam reached the threshold of his patience with all this.  The boys were frustrated that we have to wait for Boise.  Oh well.  I could tell when we got home that I wouldn't just have the luxury of going to bed.  I had left the laundry undone for the day, the dishes didn't get filled into the dishwasher and a host of other things needed to be done.  Aside from this, the kids needed something to decompress them from this very difficult day. 
     I mustered up all the energy I could find, and excitement and played a game with the kids.  It ended up turning the day around so I'm glad I went to the effort.  It would have been so much easier to go to my room, shut the door and tune out the world.  I even spent 45 min. playing the piano for them in hopes it would calm them all down.  Didn't work 100% so tomorrow, I am giving the boys a 'mental health day' from school.  I know, they shouldn't miss another day but there comes a time when the smallest thing becomes the thing to break the camels back.  School is the back breaker these days. 
     I decided that I can either sit around griping about the lack of communication we have with our doctors or be proactive and be the squeaky wheel.  Tomorrow, as much as I hate to do it, I will become the squeaky wheel.  I will start with Seattle.  I refuse to go another week in the 'mito' dark!  That has just become ridiculous.  Then I will make an appointment to see our Oncologist without the kids being there so we can have a heart to heart about what I need as a mom and a caregiver.  I know that we have a really good Oncologist, the communication just sucks so I think it will end up being a positive thing. 
    So, in the end, though it was a hard day, it's over.  Tomorrow's another day, another hope of something good happening, or not.  The laundry will sit another day, maybe tomorrow it will get done in between cleaning houses for other people.   Maybe the best thing that will happen tomorrow is that we survive it but I guess that's okay too and if Shelbie gets meningitis, I will be glad for morphine and zofran!

I hate SDS

I am about one frayed nerve away from packing up my kids and running away to find a great big rock and hide under it for the rest of our existence!  Today, I hate SDS more than I usually do. 
      We are sitting in the hospital having an infusion.  I knew it would happen this way.  I have been in the difficult predicament to decide what to do about Boise.  Tensions have been running high with Shelbie, me, and the ex.  If she didn't get the infusion today, it would be too risky to travel.  Getting it today means that she will more than likely be sick for traveling tomorrow, too sick.  I had to decide in a short amount of time if I should keep the Boise appts or cancel.  After many tears, yelling and frustration, I cancelled the appointments and rescheduled for two weeks.
        There are so many reasons why canceling Boise makes me angry.  The boys are sick every day!  Every single day, someone is sick with GI issues, bone pain, joint pain and abnormally ridiculous fatigue.  Every single day, someone is calling me from school and needs to come home.  They needed this appointment in the worst way.  I have to figure out what is going on with them and get them some relief.  There is literally no where else we can turn.  Feeling helpless is a horrible feeling.  Not only that, I am out $60 on the hotel I booked last night through priceline. 
       It's been really hard to stay calm today.  I am seriously at the end of my rope.  I think it would be easier if I didn't have the financial worries.  It might be easier if I felt like I had a team of doctors helping me but instead I have 5 or 6 that are just concerned about doing their own thing and we are getting lost in the mess of SDS.  I hate it. 
      As luck would have it, good luck,  in the middle of my melt down this morning at the hospital, my friend from the other side of the nation called just to say "Hi".  Instead, of a 'hi' she got me blabbering and crying on the other end of the phone. Probably not what she had in mind but I am glad I could talk to her.  I felt better after venting my frustrations.  She had also emailed me the words to a song she sang in choir practice yesterday and low and behold, the words were the scripture I have on the side bar of this blog.  I had no idea that verse had been made into a song.   That helped me feel better too. 
     I left the hospital for a few minutes just to calm down.  I have been talking to God all morning, hoping for understanding at least.  I know there is a reason things worked out this way.  I know there is a reason we had to postpone Boise.  I know he is working on something better for us, he is aware of us or he wouldn't have had my friend call at just the right moment in time.  All I can pray for is to preceive this situation correctly and increase my faith that in time, all these problems will be addressed.  I feel so bad that the boys will have another two weeks of suffering.  I wish it didn't have to be that way.  I guess for now, I have to hang in there.   I suspect this week will just be a harder week to deal with. I really had no idea that SDS could get this hard.
     

Sunday, January 9, 2011

In a bind

Now, we are in a bind.  Shelbie didn't have her infusion on Friday.  It was nice to have weekend without sickness after the long week of sickness we just endured.  It has caused some problems though to say the least and I have no idea what will happen this week. 
       We went in to the hospital this morning for a check and her counts are now below 60.  By the time the doctor gets these results and decides on a plan of action, it could be 11am.  That is late in the day to start a 9 or 10 hour infusion.  The nurses in Short Stay are only around until 6pm.  If she has the infusion tomorrow and they rush it, like they always do, then aseptic meningitis will set in around the time we will be leaving for Boise on Tuesday.  I can't imagine traveling with her in such an uncomfortable situation.  Then to be at St. Lukes all day Wednesday and who knows what will happen Thursday, it's just going to be hard. 
     If they wait to do an infusion, then that means we will be on icy winter roads with a girl who has no platelets.  Any accident, fender bender, any bad luck at all could be detrimental.  The infusion would have to take place when we get home Thursday or Friday.  See the bind? 
    I understand this is not something I have any degree of control over.  It does give me cause for worry though.   Furthermore, our Seattle doc finally got around to calling me with mito results on Friday but I missed her call.  I have the dumbest cell phone ever.  It rang once, not even enough time for me to take it out of my pocket and then registered as a missed call.  I called back immediately, then again, again....9 times I tried to call her back but no answer.  So frustrating, she didn't leave a message.  Hopefully, I will hear from here tomorrow but everyday this week is packed with doc appts. every single day!  With my luck, she will call in the middle of one of those visits so I anticipate it will be a long week of phone tag.  UGH!

Friday, January 7, 2011

Normally an infusion day

Well, today is the third Friday since Shelbie's last IVIG.  Wednesday, her platelets took a strange jump but today, they are well below 100.  By the time the hospital gets all that information to our Doctor and the doctor gets orders written, no infusion will happen today.  More than likely we will have to go the weekend before anything gets done.  That's a bummer.
     Perhaps it was meant to be.  Sam called me from school around 11:30 to say he was sick.  Probably a stomach virus or maybe his ongoing GI issues.  It's really hard to say today.  He hasn't been all that well all week long.  I really hope that next week will bring some relief for the boys and their constant nausea and feeling crummy.  (we meet with our GI in Boise next week)
       This afternoon, I have two kids sharing the couch and loveseat.  Sigh...Spencer went back to school today.  He is feeling better after being poked so many times yesterday so that is a blessing.  Hoping for a peaceful weekend. 

Thursday, January 6, 2011

Poor Spencer

Today was not a good day for Spencer.  He had his allergy testing today.  The Allergist said he was by far her most difficult patient.  Not because he was being 'difficult' but because he has so many severe allergies.  She had to go slower than normal and had some problems getting the amount of allergen adjusted so that she will know how much to mix in the weekly allergy shots he will start in two weeks.  It's sort of a complex process and I just confused you all with my explanation of what she had to do. 
      Anyways, Spencer ended up getting over 120 needles in his upper arms.  He was injected with all the things he is allergic to.  The pictures don't really do it justice but his arms were one big, red, swollen welt.  It was so sad to see.  He was brave, of course.  The whole procedure took over three hours so it was another long afternoon.
Only the beginning

Watching Orange County Chopper

There were 6-8 of these trays that contained the serum Spencer was injected with!

Needless to say, there were tons more than this.
Tonight, he is feeling really sick and very uncomfortable.  The welts are really itchy and sore.  So sad.... We met with our ENT afterwards and he has never seen anyone with such bad allergies and bad reactions.  He is honest when he tells me that he can't promise that the allergy shots will give Spencer any relief.  He has never had an experience with allergy shots and someone with a suppressed immune system so he is very interested to see how things turn out.  I am really hoping that it works.  He suffers so much from April until October.

Wednesday, January 5, 2011

Big Surprise

We had a big surprise of welcome relief this morning.  Shelbie's platelets took a 20 count jump!  That is so awesome.  Everything else took a big drop but the platelets going up two days before IVIG means she may not have to have it on Friday and that would be a nice break.  We will check again on Friday morning and see where things are at.  It feels good to take a deep cleansing breath...of relief.

Monday, we were asked by the Butterfly Guild out of Seattle to participate in their very first fundraiser coming up in February.  They have asked Shelbie to be a speaker at the brunch to talk about what it's like living with SDS and why research is so important.  We will not be able to afford to go so we will video the talk and they will play it the fundraiser.  The boys have been asked to chime in as well.   They have asked me to put together a video all SDS kids with music from Shelbie playing in the background. Those that are attending will be able to see the faces of all the children they will be helping as they open their hearts and wallets to a worthy cause. 

I just met the young mother who started this non profit organization this past October.  She has a 2 year old with SDS.  I am so proud of her and her efforts.  They have raised $50,000 in the last few months and hope that by the time the brunch comes around, they will have reached $100,000.  Not bad at all for such a new and young organization.  I wish I could do more to help, I wish I was independently wealthy so I could be a contributor but I guess this is not my season for doing that. 

Tuesday, January 4, 2011

This and that

Well, even though the doctor had promised to call me Monday afternoon with mito results, I still haven't heard from her.  We did go to the hospital Monday for blood work and the platelets are coming down.  Monday her count was 100.  We will head back in tomorrow and I'm guessing they will be in the 20's or 30's and infusion will take place on Friday. 
     It's a little frustrating because I still haven't heard from our local doctor.  We were suppose to have been planning surgery and a long term plan of care but that is still in the air.  Hopefully I will be able to talk to him tomorrow or Thursday. 
     In the meantime, I have 3 sick kids.  Spencer has had bad back problems but this morning he woke up with a swollen jaw.  He wasn't hit, didn't bang it, none of his teeth hurt just this swollen, sore jaw.  He also has a sore throat and his very tired.  Samuel is struggling with who knows what- more nausea, fatigue, stomach pains.  I have tried to keep him loaded up on enzymes, nexium, zofran, gas x and any other things I think might help his discomfort.  All those remedies did get him through the school day today and he was able to eat dinner so that is better.  I am glad that we are scheduled to go to Boise next week to meet with our Gastrointerologist.  Hopefully, he will have some answers for Sam. It's almost reminiscent of Spencer's gall bladder attacks. 
     Shelbie is also sick with a really bad cold.  She sleeps all day and has a sore throat and low grade fever.  It's just viral so there isn't a lot I can do for her.  This seems to be part of the pattern.  She gets sick while the platelets are being destroyed, gets IVIG, gets better for two weeks, gets sick again, platelets gone....  It's just a vicious circle and it's getting old. 
    That's what's happening in our neck of the woods.  I should have lots to report on tomorrow and not just about bad stuff.  Looking for some really good news about a fundraising project so stay tuned!

Sunday, January 2, 2011

Gearing up

Well, the break was nice but reality is setting back in.  I am preparing for a big week!  Remember the Mitochondrial test results we have been waiting for?  Hopefully I will hear about those results tomorrow.  I guess I don't really care if they have it or not, well, of course I care, I don't want them to have it!  Either way, I want it to be definitive, that's what I mean.   I don't want to hear, "Well, it's not clear if mito is what they have. We would like them to come for a muscle biopsy so that we can confirm it that way." If that is what I hear, then it will be an entire year before we ever find out for sure.  I just don't want to drag this on.   I am glad for the rest I had this past week, it has put me in a better place for hearing this news.  Being tired and worn out doesn't provide a very calming place to hear bad news.
     The next big thing this week will involve Spencer.  They have confirmed his 40 allergies through the skin test a few weeks ago.  This Thursday, they will have to actually inject him with those 40 allergens so that they can get a specific reading and formulate the weekly drug he will need to be injected with.  I don't imagine that is going to be very fun.  The first round left him really sick that night.  I can only imagine how he will feel after this.  More missed school...sigh.   I hate school more than anything!  Trying to get him caught up is worse than anything I can think of...seriously!
     Shelbie will head in first thing tomorrow to the hospital for a blood test to see where her platelets are.  It's so weird not having any idea where they will be.  She has a couple of spots of petichae but it's hard to say. 
I can say this, I hope that whatever happens this week, we will be able to take it in stride and that these infusions will begin to feel like our 'new normal', a routine that is no big deal.  That would be a really nice feeling!