A major case of the whine...

Today my Facebook status just needs to say: "Stephanie Richardson cannot stop whining, so stay FAR FAR away!"  I'm feeling FAR from grateful, and just having a really bad "why me??" day.

Why?  Oh how nice of you to ask!! And since you did, I'll share. (There is an X in the upper right hand corner to RESCUE yourself now if you like ;-) ).

Jeffrey woke this morning, as he has off and on for the past 2 weeks or so - SCREAMING! I cannot turn on his bedroom light, or the screaming escalates. I have to turn on the hallway light and prop his door open so I have enough light to change his diaper. OH great... he soaked through everything again. And I don't mean what David did and other boys do... we're talking head to toe front of him is soaked, his pillow, bedding, blankets - where does all this fluid come from? He's been off diamox for weeks now!! I get him cleaned up, but I can't clean his bed right now because he won't let me put him down.

We go to the living room and turn on dim lights.  Aly says she's shaking. I grab the blood sugar monitor to test her, but have to put Jeffrey down to do it.  Jeffrey is screaming while I test Aly - BS 70, which is okay, but is it a rebound? She's shaking pretty bad. I grab her a glass of apple juice, then pick Jeffrey back up - all 36 pounds of him. Are we out of Danonino's? Crud. (Why are there no more Danonino coupons? I refuse to pay full price!!)

Kids are throwing fits about lack of their favorite yogurt while I get a special new cereal - sprinkle Cookies Crunch. *AAACHOO*  Kay Lee walks in the kitchen and can't breathe worth a darn. Great. That whole runny/stuffy nose thing going on.  No fever, and she is all dressed and ready to go to marathon club, so I guess she has no plans on staying home.

Why oh why does this happen on the days that a front is coming through? The back of my head and my neck feel like they are going to explode at any moment! I would LOVE to do my own decompression some days it hurts so bad - I mean really, could the incision be that much worse just to relieve some of that pressure??   Yes, all 3 kids needing me to play nurse/mom/caregiver all at once when my own body is screaming to be put out of its misery.

And did I mention that Kay Lee's hair is falling out in dime size patches? It only itches on occasion, she says. The skin looks perfectly healthy. WTH does this mean?  11:45 appointment with the pediatrician tomorrow.

But you/your children don't look sick? You/he/she looks great/fine/completely healthy!
*thwap*

Yes, be forewarned. The next person, and especially doctor that says this???
I hope someone would be willing to bail me out of jail.

You were not there when Dr. R sat us down to tell us our 4 week old baby girl's stomach was twisted in half and required immediately emergency surgery - get her to Children's Mercy NOW - the surgeons are waiting! Rare condition they'd never seen.

You were not there when our 18 mos. old passed out cold one morning and didn't wake up for 10 minutes. Next episode was 62 minutes. NO ONE could tell us what was wrong either time.
Only 5 months later and TONS of tests and exploration, agony and worry did we figure out it was a blood sugar issue of unknown cause.

You were not there when after 2 years of walking, rocking, holding, sleepless nights, and begging of doctors, we are told our 2 year old boy has almost 2 cm of his brain falling down into his spinal canal and crushing his brain stem (never mind the life threatening complications that could cause).

You are not here now as we deal with the day to day complications of these 3 children's severe medical issues.  The surgery on the tummy that was supposed to be a one time fix, that turns out to be a connective tissue disorder that appears to be affecting many different systems of her body, including possible Intracranial Hypertension and Arnold-Chiari Type 0 - partial mystery as of yet.  You don't hear her complaints of nausea, catch her vomit and clean her up, hold her during the excruciating headaches and wish I could help when she says her vision in blurry, but doctors say nothing is wrong.

You don't have the constant worry if Aly will wake up in the morning, if she had enough to eat the night before, was she over active or stressed, or is she getting sick, which completely depletes her body - complete mystery #2 as of yet. You aren't here to carry her when she can't walk anymore because her legs and knees hurt, her stomach hurts, or she is shaking and can't walk straight from a blood sugar low.

And what the heck is going on with Jeffrey? Which came first - the chicken or the egg? Intracranial Hypertension or Arnold-Chiari Type I? Which caused the brain damage which resulted in the severe IGF-1 deficiency and severe Childhood Apraxia of Speech? Anyone want to help us hog tie him every night to make him get his growth hormone shot? What else is ahead?

With all this, I'm just going to say it: It is enough to have 1 child with special needs, but 3?...4?? I didn't even mention my awesome David that still needs me too! He's ADHD and needs help coping, scheduling and organizing.

So if we all look so frickin' normal and great, why is this mom so beyond exhausted? Go ahead and say it: "You ungrateful BITCH!" Well, so be it. I'm allowed a 15 minute pity party periodically.

I cannot be everywhere and do everything. The money tree is no where to be found and every inch of the bottom of the barrel is visible.

As I do every single day... I just keep praying and holding these precious babies (yes, David - my baby) in my arms as long as I possibly can.
Tomorrow... back at it.

Chiari Book Recommendation

I cannot wait to read this book.  The foreword is by Dr. Ben Carson, Director of Pediatric Neurosurgery. I've purchased it and it is supposed to be in my hands by Wednesday (perk of having a 3 month trial of Amazon Prime - free 2-day shipping!!).

I will post more on our family health situation later. However, for now I ask for prayers. Chad and I are very discouraged and confused.  Our children and myself are in pain, and we simply don't know what to do.  There is ample warmth from the sun. I'm going to bottle it and bring it out in January.

Love to all,

The Richardson's

Just say NO!!

JayHawk Dental 826 Iowa St.

I AM DONE WITH YOU!!

They bill us for the overage after EVERY cleaning, even though our insurance covers 100%. When we call them on it, they say "Oops. Our bad." No... trying to get people to pay the overage. I'm not stupid.

They made us pay up front $120 towards a procedure my son had done, but insurance covered it. They won't return the money, but leave it as a credit. (Not sure they can do that.  Going to check into this.)

Charged us $117 for them to look at another of my son's teeth that didn't seem to be coming out and causing problems - D pulled it in the office to prevent the doctor from doing it. We were there 5 minutes.

Finally this past Wed. when I was sick with 102 fever, I called to reschedule my cleaning. The front desk lady was not very friendly, but I just said that I was concerned about my hygienist that is 8 mos. pregnant, and would hate to bring germs to the office. NO response and just said - "We can squeeze you in July 7th at 2 p.m.?" Today I got a nasty letter saying that I missed my appointment on 7/7/10 (?????? rescheduled date???) and if I do it again in the next 18 mos. they are going to charge me $50. Okay fine - I read that in the financial policy - is the nasty letter with the WRONG date really necessary??? Personally signed by the dentist???

Maybe I'm just in a bad mood, but I've been with this office since I was a young child.  When this new dentist bought out the office everything became all about making money. Yes the remodel of the office is gorgeous. I don't necessarily need a TV while I'm having my teeth cleaned - been doing it for years without.

Or maybe it is the healthcare manager in me that totally and completely disagrees with their practices.  The top of the letter says "In order to help maintain a good relationship with our patients..."  Okay, am I the only one frustrated by this?? This is NOT maintaining a good relationship with me.

Couldn't the front desk lady just said on the phone : "Sure! I'm sorry you are sick. I'm happy to reschedule this one at no fee. If for some reason it would happen again in the next 18 mos. you probably know that our financial policy states that a missed or rescheduled appointment with less than 24 hours notice includes a $50 fee." I would have been FINE with that. Totally understandable.

10...9...8...7...6.... *breathe*

Yes. I will be calling tomorrow to cancel July 7th at 2 p.m. and finding a new dental office. Makes me kind of sad because I really like my hygienist.

Family Health Update

 Most of my family and friends have kept up on Jeffrey through his Caring Bridge site.  While the waiting for his surgery can be difficult on some days, I've tried to keep us active and maintain a fairly routine schedule.  Jeffrey is definitely still in pain, but I truly believe he unfortunately has learned to cope with it most of the time.  I know when he's not doing well because he gets super fussy, eyes get puffy, and he keeps wiping his face.  Every once in a while I will ask if something hurts and he points to his head and says "Lala" (right there).

Aly continues to be our mystery diagnosis girl.  We received her test results back on the growth hormone and IGF-1 - all normal.  Her liver enzymes were "slightly" elevated, but according to the nurse nothing to be concerned about. Okay, yeah right... what does it mean if they go up? What should I watch for?

Call me a bad mom, but I have to be really honest here. There is a part of me that is just beyond exhausted.  Kay Lee - rare condition requiring emergency stomach surgery at 4 weeks old.  Aly - drops unconscious at 18 mos. old and now at over 4 we only know that her blood sugar gets too low, but absolutely no clue why.  Jeffrey - has screamed in pain since birth and doctors finally found the Chiari Malformation of the brain that has already caused damage to the hypothalmus/pituitary area (we assume it is the cause) causing Jeffrey to need growth hormone shots to keep his IGF-1 levels up so he can process glucose into energy and not become severely hypoglycemic like Aly.  Really? All 3 kids having something extremely rare?  100% of the babies Chad and I produce have rare medical conditions. Well, I guess thank goodness we aren't having babies like the Duggars - can you imagine what our medical bills would be?!?!  But I'll go with what Chad said the night we got Jeffrey's diagnosis - we need to buying lottery tickets with those odds!!

Forgive me for being a Wendy Whiner tonight. I'm just tired.  I've been through my own pain and symptoms, including a trip to the ER recently for literally the worst headache of my life.  Considering what I've been diagnosed with in the past 5 years myself (essential tremors in my hands, fibrymyalgia/chronic fatigue syndrome, neurocardiogenic syncope for blackouts and extremely low blood pressure, and occipital headaches), all of which seem to decide to flair up in the spring, you can imagine I might be a bit cranky right now.  Fortunately, Dr. Di (Jeffrey's neurosurgeon) has agreed to look at my medical history and MRI from a year ago and see if I could possibly have Chiari Malformation as well - especially since doctors and researchers are finding more and more cases of Chiari being genetic.

So that's our health status - hurry up and wait - story of my life.  Next stop: Dr. C (pediatric endocrinologist) next Tuesday and will hopefully start genetic testing with Aly to get her figured out.

New Favorite Author

So I'm hooked. I've started reading Karen Kingsbury books and they have deeply touched my life.  I even joined the Baxter Family Club through Karen's website and pre-ordered "Take Four."

I have the "Let's Go on a Mommy Date" which is priceless.

Chad's mom is even more of  fan than I am, so she is the one sharing most of her books with us.  This is one she gave to Chad for Father's Day last year - read with Kleenex nearby!!Stay Close Little Girl: Words of Love for Dads

So if you get the chance, and enjoy Christian Fiction - this is definitely an author to check out.

Love and blessings,
~Stephanie

2 year milestone

He did it.  Jeffrey made it to 2 years old.  We were pretty scared for our tiny guy that arrived 5 weeks early 2 years ago.  My body was just done for.  With almost 2 times the amount of amniotic fluid I should have around this cute guy, my body gave out on him.  He was hypotonic, acidotic, and in respiratory distress. 

Praise God for Dr. Riordan!!

It was not even Dr. Riordan's night on call.  Peds was called about 4:30 for an emergency c-section.  Even the bill we received said "Ann Vangarse".   Sure enough, with our other "special" kids, instead of going home to his own family, Dr. Riordan showed up, stayed with Jeffrey much of the evening, and made arrangements to get him to Overland Park Regional NICU, where his wife is a social worker and would help oversee his care.  Besides one quick little kiss after delivery, and a wave from a distance before he left LMH, I did not get to see Jeffrey for 2 days!!  Oh how I cried my eyes out.  Chad went over with him to make sure he got settled, and was back by my side by middle of the night. Our fighter boy came home in just 7 days on Valentine's Day, nursing like a champ and gaining weight!!

Oh what a 2 years it has been... screaming fits, holding his head, low blood sugars, watery eyes, long nights, and lots of sleeping on our chest or by our side... but we've also had smiles, giggles, "Uhuh uuuh!!" ("choo chooooo!", if you don't speak Jeffrey), "ba" (ball), story times, race cars, trucks, swinging, hugs for the puppies, and lots of snuggles.

So for now we enjoy the good times, and pray for his health and what is ahead.  Jeffrey was average height and weight and appears healthy to Dr. Riordan for his 2 year check up.  We are still waiting on the MRI results (PRAY we get them in the next couple days... I'm not good at waiting!!), and see the endocrinologist again on the 16th.

Happy Birthday to my favorite J-man!!

He might be giant!

Okay, not really.  But the doctor called today to say that Jeffrey passed his growth hormone replacement test.  Before shots his IGF-1 was at 39 (normal is between 45 - 222 however most kids average 80-200). After growth hormone shots (which stimulates the liver to produce IGF-1) his IGF-1 was at 109.  So we know that Jeffrey can produce IGF-1, he just needs help.  I'm still learning about why he needs help this way, but at least he does not have to move forward with IGF-1 shots, which would be twice a day.

Good news: Jeffrey passed.

Other not-so-good news: Doctor wants him to take growth hormone shots 7 days a week before bed.

Jeffrey's blood sugar has not dropped below 70 since he started the shots!! So our #1 goal seems to have been achieved.  Now for the remaining questions the doctor (and we) still wants answered, and our own questions.

Doctor:
1. Why is Jeffrey of normal height, but IGF-1 deficient? (If I didn't explain before, IGF-1 is what helps our bones, muscles, organs, pretty much everything grow!)
2. What is causing the IGF-1 deficiency?

Our questions:
1. How long will Jeffrey need to take these shots or can we predict that yet?
2. Will this accelerate his growth?
3. Do we need to watch for high blood glucose now?
4. What is the long term prognosis?
5. Should we still pursue genetic testing?
6. What about Aly?

What I know for now is that I'll run to Topeka to Dr. C's office to pick up another sample GH pen tomorrow.  The nurse submitted the application to the pharmaceutical company and our insurance company.  This is a long process because GH injections and the dose that Jeffrey is on will cost $365 for 6 days worth of injections or about $1200 for 20 days (a pen with more meds in it).  Chad's comment today was "so we'll do the injections until he can take it orally." The answer is no.  Growth Hormone is not absorbed in the intestines (if I understand it right), so he may have to have shots until he is done growing or at least of average height - depending on control of his blood sugar. Some do continue into adulthood.

I do feel some relief in that it does seem that with this I am less likely to have to constantly worry about his BS.  Obviously there are financial concerns, especially if you consider the cost of the shots and if we need to pursue the genetic testing which is almost never covered by insurance.  Top this off with we still don't know about Aly. Our suspicion is that her low BS is caused by a different hormone malfuction, but we don't know what that will entail. 

So we just keep putting one foot in front of the other, praise God for the small successes and answers like we received today, and keep doing our best.

Jeffrey's MRI still planned for Tues. Feb. 2nd
Annual physicals for both Aly & Jeffrey on Thurs. Feb. 4th
Both Aly & Jeffrey go back to Dr. C (peds endocrinologist) on Tues. Feb. 16th

The J-man's update

So I'm not real thrilled about the whole hormone shot situation.  The nurse gave us instructions on how to administer the shot. Prime the pen, put a fresh needle on, dial out to the appropriate dose. Clean J's bottom, let it dry. Push the button, jab it in, count to 5, and take it out.  Sounds pretty simple. So Thursday night, this is what I did!  Jeffrey is such a trooper - he just watches every poke he's ever gotten and never even flinches.

Friday night's injection comes along, and I have the instruction pamphlet out to make sure I'm doing it right again. Here are the exact instructions (without the babble):

• Push the needle under the skin...
• Press the Push Button all the way, keeping it fully depressed with the needle under the skin for at least 6 seconds to ensure the full dose has been delivered.
• While keeping the Push Button fully depressed, remove the needle from the skin.
• (Next page over regarding removing the needle from the pen) The dose scale will reset to 0.0 and the pen will be ready for the next use.

So, not exactly as the nurse explained it, but pretty close.  I follow the instructions, but as I'm pressing the button, the dial starts going down!!! Uhhhh.. um. So I pull the pen out thinking "what the hell just happened?? That didn't do that last night!!"  Then I realize that just pushing the button - nothing is coming out.  As the dial goes down, the hormone comes out!! Okay, so maybe this is a "Duh!" moment for all my nurse friends and maybe some others of you - but how was I supposed to know??  The nurse SHOWED us how to do the injection, but when she practiced on the dummy, the dial didn't go down. The instructions say "Press the Push Button" and on the NEXT page says something about the dose scale resetting to 0.0.  Goodness.

In my defense, I did wonder on Thursday night why it did not appear that there was any LESS liquid in the pen, but assumed I was doing it right.  So now it is 8 p.m. on Friday night with no contact available to figure out what to do.  J's only gotten a partial dose (and I wasn't even sure how much) and for sure did not get ANY the night before.

Well, now that we knew how to do it, we just waited until Saturday night, gave him the correct dose, and did the same again on Sunday.  This morning when I spoke with the nurse (sub for Dr. C's real nurse), I just told her to let the doctor know what happened.  Hopefully it was enough to give a valid result on today's bloodwork Jeffrey had drawn.

After all that, my gut feeling is the hormone injections were not what Jeffrey needed. His blood sugars were still low, and he had headaches all weekend (which he had before the injections).  It appears that my sense of urgency in this matter is not translating well to the doctor or nurses.  I work tirelessly to maintain his blood sugars above 70 (sometimes getting up 2-3 times a night to check him and have to wake him to feed him).  Jeffrey screams, holding his head, and is so frustrated with everything right now.

Nothing makes him happy - and yes I know... could it just be an almost 2 y/o tantrum?? He has thrown these "tantrums" since he was about 3 months old - flailing his head, hitting his head, arching his back, and screaming... for HOURS.  Tylenol never worked when he was really little. Motrin sometimes takes the edge off.  His right eye waters randomly. He is extremely sensitive to light when it is really bad. A sunny day will set him off.  The only thing that seems to help is for him to get a nap, or some sleep.  I say that, yet some mornings are a bear - going from darkness to light and he acts like I'm stabbing needles in his eyes.  There are also times, like in this picture, during periods when he's at his worst that his eyes look puffy.  Not just in the morning, but all day. This picture was taken late afternoon.  Maybe I'm over-thinking everything. *shrugs*

February 2nd at 7:30 am we will check into KU Med for him to have an MRI of his head (with and without contrast).  My hope and prayer is that this will provide some answers.  J's IGF-1 levels are consistently low - last check was 43. Most children hang out in the 80-200 range.  Something is wrong with my J-man, and I pray for patience, pray for God to guide the doctors, nurses and healthcare practitioners to work with us with a kind heart, and that we can get Jeffrey healthy.

"They that wait upon the Lord will renew their strength. They will soar on wings like eagles. They will run and not grow weary. They will walk and not be faint." Isaiah 40:31 - my favorite verse that I so often forget to embrace.  God's energy. God's plan. God's time.