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.

Giving it a try - Growth Hormone shots

I mean seriously... could there be a cuter Jeffrey in Thomas clothes with chronic bed head that I simply cannot get to lay down? NO!

Jeffrey had MORE lab work this morning to have initial numbers on his growth hormone levels. (Poor guy - it took 4 pokes and 2 lab techs to draw his blood!! He didn't even flinch!!!) It is his IGF-1 that has been low on all previous blood work, and this is what the doctor believes is causing his low blood sugar. What has the doctor stumped is that Jeffrey is well within the normal range for height!

So we are giving growth hormone shots a try to see if this will help keep his blood sugar levels up. One shot for 3 nights, and then we will go have more blood work done Monday to see if it is helping. We will have an idea simply by my monitoring his blood sugar at home, but the blood work will give us an idea if his IGF-1 level is up.


So being the obsessive mom with a Masters in Health Administration that I am, I asked for a copy of his most recent blood work. It shows that his urea nitrogen (BUN) levels are high, which could be a sign of kidney issues. I called the nurse back and she said if the doctor didn't say anything about it to not worry about it. Well, why did it flag as out of range if there is nothing wrong? Next I put a call into our local pediatrician. Thankfully he has been amazing with our family for over 13 years. He so graciously looked at the test results, called me, and said that yes the BUN does appear high. Sometimes that can mean a lab error. However, just to be safe, let's retest. I hate to have to put Jeffrey through another poke, but we'll go do that tomorrow.

I've been doing a lot of reading tonight. I am a complete nerd and love doing research and reading medical journals. It is scary to read so many possibilities that are out there with Jeffrey's symptoms, so that is why I stopped and am now blogging (journaling). We'll go with the flow for now. It will take 2 weeks to get blood work back, and by then we'll be ready for the MRI appointment.

Our Aly-oop has kinda been on the backburner since she so graciously has been stable with her blood sugar (not that she can control it). If we ever figure out what is going on with Jeffrey, maybe it will give us more clues as to what is going on with Aly. She has never had many of the symptoms that Jeffrey has, so we've been successful just monitoring her blood sugar and feeding her. But she will be the center of our world this weekend as we celebrate her 4th birthday!! Watch for pictures of this amazing princess birthday cake!

Mid April update

Have I mentioned?? I LOVE SPRING!!

*whew* I cannot sleep, yet during the day my body screams for sleep. Yeah it has been a rough month for me. Hysterectomy went beautifully on March 25th! I cannot even begin to tell you how excited I am that is over - no more monthly female issues after 22 years of hell!!

So then I came down with clostridium difficile or c-diff as it is referred to. I'm not sure my bowels will ever be the same. I will spare the awful details, but basically a bowel infection that kept me in the bathroom for about 6 days, 2 of those days hospitalized for IV fluids and medication. That was back starting March 29th, and while I'm not in the bathroom ALL day anymore, my abdomen still is tender and bowel function is painful and certainly compromised.

With that, concern about what is going on with my heart (which they found 2 days before surgery, but hopefully just a murmur that is not a problem), fibromyalgia is out of control again with the weird fluxuations in weather and rain, my days are sometimes long. I am fully determined to get healthy again as soon as possible. This is embarrassing, tiring, and feels like my life is on hold until...

On much better notes: Chad's brother and sister-in-law announced that Harley (their dog) is going to be a big brother!! Yes, my kids will finally get a cousin around the end of October!! Woohoo! We are beyond excited!

David is currently one of the managers for the West track team. He seems to really be enjoying the fellowship. This guy also has started receiving mail from universities all over for summer camps due to his amazing ACT score back in February. As a 7th grader he already scored the same as Chad did in high school (but we won't rub it in - Chad is very smart). Needless to say, the recruiting begins! We will be attending an awards banquet in June to recognize him as one of the top students in Kansas.

Kay Lee will be attending Kindergarten round-up on April 30th. I cannot believe how time flies and it is already time for this! She is so smart, talented, friendly, and READY. Kay Lee came to us last weekend and said "Mom, Dad, look! I tied my shoes!" Ready, indeed.

Aly continues to be her sweet, spunky self. I do not know how else to describe it because one minute she will curl up with you sucking her thumb and love on you. The next minute she is running like a silly girl and all I can do is PRAY that she does not run out in front of a car. I cannot keep up with her!

Sweet boy Jeffrey could not get any cuter. He has started pointing and babbling so it sounds like he really has something important to say!! It is adorable. He is literally running with his little push toy, but is still not confident enough to walk on his own.
The picture is the 3 youngest having a bonding moment right before bedtime. Jeffrey started clapping and hollering "DADADADA", so noting that mom and dad started laughing, the girls joined in on the silliness.

Spring Break

Oh my goodness! Could God have provided better weather for those of us not traveling for spring break? I think NOT! We are enjoying being outside almost all day going to the park, play dates, the St. Patrick's Day Parade and taking the dogs for walks.
Kay Lee is really missing school, but got to see her friends yesterday. We will probably see them again this week at the park. Aly is anxious to see Andy sometime this week as well. David met up with a large group of friends for an afternoon on Mass. Street - one of 3 guys in a group of 11. While a bit embarrassed, he did great following mom's guidelines of letting me meet his friends before he got to hang with them for the day.
CHEERS to this weather continuing with a touch of rain here and there for my flowers and grass!

Jeffrey's surgery

Jeffrey came through his surgery like a trooper! He such a solid, chubby little guy that it took nine (9)!! tries to get his IV in, and then they had to put it in his head :-( Eventually he got restless enough and pulled it out. Luckily it was about 2 minutes before the nurse was going to take it out anyway.

So far this afternoon he has just had tylenol, not codeine! Here at 7:30 p.m. he is playing basketball and blocks with his dad.

Thank you for all your thoughts and prayers!
~Richardsons

Jeffrey

Everyone has recovered except Jeffrey. He will have tubes put in his ears and have his adenoids taken out on Thursday, Feb. 26th. Please keep him and his doctor in your prayers that all goes well!!

Illness status

Chad - probably tired, but seemingly healthy

Steph - fever, tummy ache, exhausted

David - healthy

Kay Lee - healthy

Aly - Must be on another growth spurt - fussy

Jeffrey - fever, pulling on ears and chewing - so teeth or another ear infection

Is this not getting ridiculous this winter? I simply cannot get everyone healthy at once!

I am so ready to open up the house and let SPRING in!!