Blood, Gut, Hormones and Teeth: These are the updates for this week!

Blood, Gut, Hormones and Teeth: These are the updates for this week!

Disclaimer:  Unfortunately, this post has NOTHING to do with zombies.  Disappointing, I know.  

However, this post has everything to do with updates from Elizabeth's appointment this week!  

Hematology (Blood):

Elizabeth had her first Iron Sucrose infusion at the Outpatient Chemo Infusion Center at the Hematology Department at Children's on Monday (2/15).  She will have these infusions for the next three weeks on Monday, and after that we will take a look and determine how often, or for how long, she will need to continue getting the Iron Sucrose infusions. Her doctors determined that these infusions would be helpful to help to keep her hemoglobin counts stable. Recently, her hemoglobin counts have been dropping, and she also has iron deficiency, and both of these are occurring, because she has a bleed somewhere, we just don't know where she is bleeding from since it is internal. The hope is that these infusions will keep her hemoglobin counts stable and high enough that she won't need blood transfusions at all or as often. 

Her first infusion on Monday went really well.  The nurses in the Infusion Suite are all very nice and friendly.  They blew bubbles, sang, gave her a present, acted silly, and basically did whatever it took to help me keep Elizabeth happy during the infusion. Elizabeth tolerated the infusion very well, and she had no adverse reactions, which is wonderful. So, we will return next Monday (2/22) for her next infusion at Children's Hematology/ Oncology Unit.  

The only bad news is that her hemoglobin did drop this week, and it dropped from 9.7 (Feb 8th)  to 7.8 (Feb 15th).  This is just further evidence that she does indeed have a bleed somewhere. The lab work was drawn and processed before she had the Iron Sucrose infusion on 2/15, so we are waiting her blood results this coming week (2/22). If her hemoglobin counts are still low, then she will need a blood transfusion next week, as well as her Iron Sucrose infusion.  

I-Care (Gut):

The I-Care division at Children's is the Intestinal Care and Transplant team in the GI division at Children's in Pittsburgh.  They see Elizabeth due to her intestinal failure, GI motility issues, and need for TPN feeding via her Central Line. I love Dr. Alissa in I-Care.  He and his staff are simply amazing.  We have lots of good news about her gut to report.  

As I explained in my previous post, we started a new drug with Elizabeth in December called Reglan. It helped to kickstart her gut to get it to work!  This was nothing short of amazing.  Even Dr. Alissa is super pleased, and quite a bit surprised, that it did actually work.  As of right now, she is up to 40 ml/ hour/ 24 hours in her G-Tube, and just two months ago, she was not tolerating anything into her gut.  

Since her gut seems to be working, we are going to relish and cherish this progress.  This means that we can start to wean her body off of the TPN feeds, which is great.  She is doing so well with G-Tube feeds that she technically no longer needs the calories from the lipids that she receives 4 days a week in her TPN. So, we are going to pull the lipids from her TPN.  This is excellent, because the lipids in her TPN really cause the most damage to her liver, and by eliminating the lipids it will give her liver a much needed break.  She is also going to be receiving a reduced volume of TPN throughout the day, and she will only be infusing her TPN for 20 hours instead of 24 hours a day, which gives her entire body a break!  

As far as her g-tube feeds, she is also receiving a high enough volume to receive a daily break from g-tube feeds!  So, starting this week, she will work up to a 4 hour break each day from her g-tube feeds.  As we increase her daily break from her g-tube feeds, we will also be increasing her rate of feeds each day by 1 ml, just as we have been doing.  So, this means that today, for example, her rate for g-tube feeds is 39 ml per hour, and tomorrow her rate will be 40 ml per hour, and so on.  As long as she can maintain her blood sugars within normal levels during her breaks from TPN and her g-tube feeds, then we can continue to give her daily breaks from feeds.  It will be true freedom for her to have, even a little, time each day that she will be disconnected from lines and tubes and pumps!  

The breaks from TPN and g-tube feeds will occur simultaneously.  So, each day, she will not be receiving her TPN or g-tube feeds for the same 2 or 4 hours each day.  If during those breaks we have a blood sugar drop/ hypoglycemic episode, then I will treat her as recommended by her doctors.  I can either treat her using a medication called Glucagon to bring up her blood sugar, or I can simply restart g-tube feeds.  Hopefully, she can tolerate the breaks, and experience freedom from being connected, and even for a few hours have a taste of that freedom.  I can't even begin to tell you how exciting and hopeful this all is for all of us. We will see I-Care next month, and hopefully we have only more good news to report. 

Oh, one more thing, Elizabeth's growth chart looks incredible!  She currently weighs a little over 31 pounds, yes, 31 pounds.  We finally have broken an all time weight record.  She now weighs 10 times as much as she did when she was born, which is amazing!

Endocrinology (Hormones):

We have been following with Dr. Sorkin in Endocrinology for over a year due to suspected growth hormone issues, as evidenced by changes in her brain MRI to the corpus callosum and pituitary gland. We also follow with Endocrinology due to her Ketonic Hypoglycemia for treatment and monitoring.  Since Elizabeth is growing, and her Ketonic Hypoglycemic is being well managed by closely monitoring her blood sugar, and treating episodes with Glucagon, it may be possible to follow with Endocrinology once per year, or as needed, instead of every 6 months.  We won't be able to know for sure if this will be possible just yet, but it may be quite possible.  Currently, we have to wait for some labs that she will have drawn on Monday at Children's regarding growth hormone, pituitary and adrenal gland function, as well as her brain MRI on the 29th before we can know for sure if we can change how frequently she is monitored by Endocrinology. So, we will keep you updated on that!

Dentist (Teeth):

Celtan and Elizabeth had dentist appointments today.  We see Dr. Kobil in Butler, and their staff is amazing.  Our friend, Janet Peters, is a hygienist there, and it is always nice to see her when we are there.  I think she is Elizabeth's favorite, especially after today, because today she gave Elizabeth a Princess Wand after her dental exam.  Elizabeth really loved it.  Elizabeth and Celtan both were given excellent reports from the Dentist: no cavities and praise for clean teeth.  So, we are good in that department!  

 

What's Next:

This coming week, we have some additional appointments. Next Monday (2/22), Elizabeth has her second Iron Sucrose infusion at Children's, and then she has an equipment evaluation at the Children's Institute later that afternoon.  We need to determine if a new adaptive stroller or wheelchair will be the best next step for her.  So, we will be sure to update you on that, as well. 

Team Elizabeth Jeanne is also excited to give back this week.  On Saturday, I am helping to serve at a dinner for the families at the Ronald McDonald House at Children's.  My friend, Meghan, is hosting the dinner, and asked me if I wanted to help about a month ago. I absolutely said, yes! We don't qualify to stay at the Ronald McDonald House when Elizabeth is at Children's, because our address is about a mile too close for their distance requirements.  However, I have met many families during our stays at Children's who stay there, and I know what a wonderful service the Ronald McDonald House at CHP is for those families.  So, my sister Holly, Jason, Celtan and I put together 65 toiletry bags and 65 sibling activity kits to give to the families who attend the dinner from Team Elizabeth Jeanne. A special thank you to my cousin, Ashley Ciafre for ordering the bags and sunshine tags, and to Holly, my parents, Gram and Pap Kepple, and Aunt Debbie for helping to gather up items to put into the bags.  I'm so excited to be able to help out with the dinner, and to be able to give back.  It's such a great feeling to donate, give back, especially when so many people have been so generous and kind to us.  Elizabeth watched us with a clear look of sassy disapproval as we stuffed bags.  We must not have been working to her liking, but I think deep down she was just as excited as we were.  At least, that is what I'm telling myself. 

 If you like these updates, or the way that I write, I recently started a personal blog called "A Love Spiced Life." If you're interested, you can find it at www.alovespicedlife.com. 

Just for cuteness factor, here's a picture of Elizabeth looking at the Valentine that her brother made her, and cute pic of our growing princess!  We hope you have a wonderful day! Until next week, thank you for being a part of Team Elizabeth Jeanne.