Special Kids

Tube Changed!

Yeah! Praise the Lord! They had an open slot in the IR today and got Kierra’s new J/Gtube in. She did very well and didn’t need to be totally ‘put under’ but only sedated. Her IV was leaking, so they got a new one started as well while she was a little more relaxed. Now we are slowly increasing her feeds every 3 hours until we reach our goal of 47 ml per hour. We are at 10 ml right now:) She is sleeping again finally. She was awake all night the last three nights. I think she just wants to go home to her own bed. Thankfully she took a nap today, now tonight she is sleeping again.

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The wonderful thing about this cold snap that has everyone shivering is that Steve got to have off work yesterday and today! That made me very happy! I even got to take a long nap this afternoon…bliss!! Personally, the cold cold outdoors felt very invigorating and brain clearing to me on my short walk to the car πŸ™‚ Nope, I did not have to stay out for any long amount of time.

Steve headed home again tonight. Hopefully we can be discharged in a day or two and celebrate being together again. I miss Kobe! πŸ™‚ It’s so much fun to talk to him on the phone now that he’s old enough to respond.

Guess what else? I got my cracked lenses fixed in my glasses yesterday! They have cracked twice now, and I haven’t even worn them much. Kierra likes to reach up and box them with her long arms, but I didn’t think that would be hard enough to crack them. Maybe they are weaker then I thought. This time I got a more shatter resistant lens.

Just a little secret… Lens Crafters was going to charge me $300! for replacement lenses. Vision Works only charged me $90…and did it in a few hours! I was highly impressed! I think they did a super job of getting my eyes focused in them too, since the last lenses made my eyes go weirdo. I can actually wear them without feeling tipsy! I guess it just takes coming to the hospital, having a great husband to watch Kierra,and a Vision Center close by Β to give me the nudge to get them fixed. LOL! Oh! And I think this vision center did a MUCH better job then Sears where I got my glasses πŸ™‚ So …good things come to those who wait πŸ™‚

Yesterday, a friend texed me the amazing thought that God watched his Son suffer to death and didn’t do anything about it…because He loved us SO MUCH! So don’t think for one moment that God does not hurt with us when our children suffer also.

Last night, a Pastor stopped by on His rounds, and in our conversation, he mentioned the exact same thing! That God really DOES care that we live in a hurting imperfect world. He sent Jesus to this messed up place for US! Isn’t that just the most amazing Love and Grace!

It was a wonderful confirmation that God will walk with us through whatever we face in life.

Thank you all for your prayers! This has been a wonderful day! πŸ™‚

Stay warm.

Special Kids

Quiet Day At Dupont

We had a relaxing quiet day today. Kierra calmed down majorly after her feeds were discontinued and her belly had time to empty and heal. She went from thrashing and crying to laying calmly and resting quietly.

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The GI Dr. and kidney Dr. came through on their rounds today and decided it would be best to change her Gtube to a G/Jtube. That will make so her feed can bypass her stomach ,hopefully causing her less pain,discomfort and vomiting. Last night, they tried to start her feeds at a super low rate of 10 ml an hour only to have her vomit after 2 hours, so we know that she cannot tolerate formula in her belly right now. The only other option for hydration is through IV. This tends to make her retain more fluid so the kidney Dr wants to discontinue her IV asap.

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We waited all day for a time slot for her procedure to open. Nothing did…they were booked full. Tomorrow is full as well. We are hoping and praying for a cancellation so she can get off her IV fluids and start with her formula feeding again. She will have to be sedated for the Jtube placement since it requires threading the tube down into the intestines. She’s pretty good at rockin and rollin when it comes to someone messing with her belly, so I’m guessing it will take a fairly large amount of sedation.

Today we went for a little walk down to the family room, and hung out in our room. There is alot of RSV etc in the hospital right now, so I wasn’t too sure about taking her out, so I covered her Kid Cart with a blanket. No children under 10 yr. are permitted on the floors right now through the flu season, so it was a nice quiet walk πŸ™‚

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She has been awake for most of 48 hours now. This afternoon she opened her eyes and laughed and talked and gave kisses πŸ™‚

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I think her gazes toward heaven just melt the heart of her Creator and make the angels sing.

She finally fell asleep tonight and is totally cloncked out. I am so thankful!! I love to see her at rest when she so desperately needs it!

Prayer Requests:

1. That she could have her tube changed tomorrow sooner rather then later πŸ™‚

2. That she would not catch a nasty bug here in the hospital.

3. That all the IV fluids wouldn’t cause her to retain too much fluid.

4. That she could be calm for the tube change and it would go well.

I talked to Kobe today. It was so much fun and he sounded like he was living the good life! πŸ™‚ I’m so thankful he has a good safe place to stay and that he is well cared for and loved while we are here!

Thank you all for your prayers!

Special Kids

Admitted

After Kierra vomited at 4:30 this morning…again and cried uncontrollably and was totally miserable, I emailed our Good Doctor. He so kindly called me on a Saturday and we decided with all Kierras ‘glitches ‘–vomiting old blood, refluxing badly, lower heart rate when sleeping, cold sweets, and generally becoming more miserable, our best choice was to take her to Deleware where a wonderful group of Drs could work together to make her more comfortable.
We packed our bags and cleaned up the house and fueled up with gas and Coke and headed out, dropping Kobe off at his beloved. Merv’s House πŸ™‚
6 Hours of sitting in the ER and now we are settled in our room. Kierra has cried most of the time if she isn’t sleeping. She is finally relaxing after plenty of Valium and giving her belly a break from her feeding. As long as she is on IV, she won’t dehydrate. Her afternoon was very traumatic with abdominal and chest xrays, labs, IV started, urine sample collected,and Kayexalate medication that gave her awful gas and a huge blowout but also brought her potassium levels down nicely.
They want to do an ultrasound on her abdomen to check for any other major blockages or obstruction in her bowels /intestines. Hopefully on Monday we can see the GI. DR.And perhaps get a feeding tube put in that bypasses her sensitive stomach.
Prayers for her comfort! Also that her electrolytes would continue heading in the right direction!
Now I will quickly post this before I get interrupted again! πŸ™‚

Special Kids

Two Little Wildflowers

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Kierra met another little friend today that is diagnosed with the same disorder that she has. Kayla is 2 yr. old, almost three, but she easily held her own beside Kierra πŸ™‚Β Her parents traveled from Indiana to do some very informative testing at LGH here in PA. She had just returned from an MRI when we got to the hospital to see her, but she still smiled and wiggled and blinked her eyes coquettishly…just like Kierra πŸ™‚ They even locked their arms together for awhile and lay there happily beside each other, while we parents visited and loved watching them.

There is a marked difference between the two little girlies though. Β Of the 27 known cases of Yoder Dystonia, this little Miss Kayla Β has no kidney disease at this point. Not even a trace. Last year, The Clinic Dr.s and scientists met with more Dr.s and scientists that were discovering more children with Yoder Dystonia, but were calling it Hershberger Syndrome instead. They pooled their knowledge together, and discovered that little Miss Kayla had a different mutation to one of her genes then any of the other children did.

To make it all a little easier to understand, good Dr. S. explained that while Kierra and every other YD child has a mutation in Β genes that affect the kidneys and the brain, Kayla only has a mutation in the gene that affects the brain. These genes are right beside each other in our gene pool, so he described it like shuffling a deck of cards. Sometimes the cards stick together. That’s what happened to all the children except Kayla. The kidney gene and the brain gene both became mutated or ‘stuck together’ and were affected. Kayla’s gene Β broke off before it reached the ‘kidney mutation point’, so her kidneys look pristine.

Isn’t that just amazing!?!

Now they need to find a child with only the kidneys affected and not the brain! πŸ™‚

So I’m not sure what all this means in finding help for these kids, but it is WAY ahead of where they were a few years ago. Knowledge is power. At least I hope so in this case πŸ™‚

These children’s Cerebellum in their brain deteriorates at a very high rate of speed. Kierra’s may perhaps be at the deterioration stage of an 80 yr. old. An MRI would likely show more, but this is an estimation from other research. Maybe that is why she has trouble sleeping πŸ™‚

They are hoping to have a reunion for all the Yoder Dystonia children this summer sometime. I am very excited about it! They also hope to have more information to share with all the families. Maybe I will discover then if my memory served me well or if I got it wrong in my brain’s computer as i listened to the Doctor’s knowledgeable explanations πŸ™‚

The more medical term for YD is called NCS. This stands for

Nephr (kidney)

Cerebelo (brain)

Syndrome.

That makes more sense to me as well πŸ™‚

So maybe technically we would say that Kayla has Cerebelo Syndrome and Kierra has Nephr Cerebelo Syndrome πŸ™‚ Either way, they were very much alike and very much different. Two lovely little sweetie pies.

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On the way home we picked up a bunch of medication refills for Kierra including her upgraded brand of pepcid. She is still having problems with a GI bleed and is still spitting up blood sometimes. We are still waiting on some test results. Poor girl. Her stomach muscles must be getting worse, since she is vomiting 3-4 Β times a day. Thankfully, it’s not more then a few mouthfuls, but it’s still traumatic and uncomfortable and yuck for her.

We are hoping to change to a different style feeding tube that will bypass her stomach and take her feed straight down to her intestines so she doesn’t have to vomit as much. She is loving her bed and her new Bird and Flower bedding very much these days!

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