Thursday, January 28, 2010

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.

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

Monday, January 25, 2010

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.

Thursday, January 21, 2010

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!