Life with a boy and his Hypoplastic Left heart Syndrome, Also a baby girl and her Tethered Spinal Cord Syndrome, Neuro Endocrine Cell Hypoplasia, Lumbar Hemangioma, and Two Super Sister's!

Monday, October 13, 2014

Wow what a week!

Some weeks tend to make me tired... I take that back, exhausted, discribes it even better. Tuesday Gator woke up to his PICC dressing being ripped off in the night. We had to have it replaced immediatly, Wednesday I took Ry and Eden to their well check visit. Both are growing beautifully. Ry was incredibly embarrassed about the whole thing. Tinkle in a cup???? Are you kidding me??? This is sick people.. She survived. Eden got 4 immunizations. Bless her little heart she was not happy about those either. Then, just because we always need a little more excitement in our lives, I had Doc Mashkuri check out a big ol bump on Gator boy's inner thigh.. We had to drain that sucker and it was as painful as it sounds. We cultured it to see exactly what we were working with and he was put on an antibiotic. The next day was clinic and when we talked about the offending hunk-a-zit-thing it made Tx squirm and check the cultures. Staph!! We changed his antibiotic to a stronger attacker. Friday morning Gator was bothered by chest pain for three hours. What an annoying thing, especially since he had been planning a super bodacious dude camp out with Papa C, the uncles, Wells, and daddy. He must have conviced himself that he was fine because he had a really great time till the next morning at breakfast the pain came back. I brought him to the hospital while Eden and Ry went to Idaho and May and daddy hung out. Gator was admitted to the hospital. He needed to be watched a little closer. I had a ticket to a conference I was super excited to see. It was such a refreshing celebration of trials and it buoyed me up spiritually. Meg Johnson, Hank Smith, and John Bytheway were the speakers.. The only thing that went wrong is... During the very last speaker who happen to be Hank Smith I had my phone on silent but down near my purse so I can watch it. It starts to ring and it's the hospital. About 49 different thoughts went through my head all at one time.. He's really sick, he's dead, they have a new heart for him.. You know little things like that. I contemplated not picking up. I knew how rude I would look. I was on the fourth row right in the middle.. I just had to make sure all was well.. I got up and ran out.. Mortified!!!! They had a few questions about medications and wanted to make sure everything was good.. Whew!!! 
I waited in an incredibly long line to apologize to Hank Smith for running out like that. He was super nice and wasn't even mad. 

Sunday was perfect.. Gator and I and miss MayLee the wiggle. Went to the branch at PCH.. A-mazing!!!! 30 minutes is the whole meeting. Pfffffsshhh! I could do that time frame anytime. They don't need it to be longer because it's like taking the spirit and magnifying by like 10,000. I have already professed that I am sure that angles walk the halls of the hospital. Well, where do you think they go for church? Yeah it's that good. If you are ever at the hospital on a Sunday.. Starts at 10:30 and you may come in your PJ's and slippers. They have never turned Gator away for wearing his. Gator also had a primary lesson from his amazing new primary teacher Sister Christianson. Bro C also came up. She brought Gator's primary program part. We are coming up with a way for him to be included even if he is in the hospital. Don't you love technology? With a CD of the songs to practice we are excited to be part of it. It is amazing to be thought of. Gator had another Bortisamib treatment. His PRA went slightly up..69... We can still rejoice because it's not all the way back up and hopefully the meds will knock it back down again. 
We also found out his BNP went up to 1000 from the last check it was 600. 

Question: What is a BNP blood test, and should it be done on everyone with heart failure?

Answer: A B-Type natriuretic peptide is a naturally-occurring peptide produced by the body. A peptide is a group of amino acids, the building blocks of proteins, and this particular peptide is secreted in response to certain stress; this one in particular is the stress in the heart, particularly when the heart is stretched.

One of the hallmarks of heart failure is when people hold on to too much fluid. When they hold on to too much fluid it begins to backup and literally stretch the heart. That extra stretch causes the cells of the heart to excrete BNP and that can be measured in the blood.

And the higher the BNP generally the more fluid someone maybe holding on to, although it does vary a little bit from person to person. But that why it can be useful in the setting of somebody being short of breath to distinguish whether the shortness of breath is due to fluid being held on because of heart failure or whether it's due to lung disease.

We think the reason Gator was having chest pain was because of the heart swelling. 

Today we relaxed.. He was stoked because Doc Everett reduced his sodium ban. Can you guess what he chose?? Top Ramen. He slurped and yummed through the entire bowl. Auntie Pres had the girls today, she even did all my laundry. Bless her! Tomorrow she goes to spend the day with Gator while I am with the girls... Daddy is up there to hang with his boy in the evenings.. How he managed to organize life, work, church, and still be so dang amazing is beyond me. Sometimes I feel like bowing down and saying "I'm not worthy, I'm not worthy" but I shouldn't give him a big head and so I refrain. 

Pray the antibodies will go down again and also,  in the midst of grief a family will choose organ donation. 

Sunday, October 5, 2014

The ultimate catch up 3rd edition

When last we left off things were somewhat status quo. As the weeks have progressed Alex's body has declined. One indication of this is where his liver sits in his abdomen. If it's lower in his body that's bad.. It is normally measured from rib cage to bottom of liver. It is more prominent, easy to find and measure when it isn't happy as well. Alex usually measures a 5-6... Two Thursday's ago, it measured at 8-9. We upped his Diurel (diuretic) to the highest dose. This with instructions to have a blood test on the next Monday to make sure his body wasn't getting too dry. Also with a caution that we have upped all his home meds to maximum and the next step would be hospital intervention. In other words, it would be wise to keep a bag packed. We were also waiting to hear if Alex's body had responded to the treatments to lower his antibodies. Nothing had worked before. I was still hopeful but not 100% committed to believing this time would be different. Monday rolled around and our nurse Dylan came to draw blood. I waited for a couple of hours for the results. When I couldn't wait any longer I called. Everything was still pending. It wasn't until the next day that we heard that Alex's kidneys weren't happy. We backed off of the diuretcs. Still waiting on the PRA (antibody panel). On Wednesday we got the best news.... Alex's antibodies had indeed dropped, from 85 to 61. Of course I had a million questions but there weren't any answers yet. Our team knows how much I worry and wonder and wanted to deliver the good news but hadn't had even a minute to plan beyond telling us. Bless them... I jumped around like a lunatic. I squealed, called daddy and all around was fit to be tied, in a good way. 
 Last Thursday at clinic, we rechecked his kidneys which were back to being happy. We thought he could have freedom to eat sodium but he ballooned up. 
Those are some puffy eyes.. Back to watching his salt intake. He was so miserable that all he wanted was to sleep off his sodium hang over. 
We are doing daily weight checks in order to see if he retains fluid. If so we can cut back even farther on his sodium. Even though it's the only thing that tastes good right now. Good thing Heart Momma Jill had my back and found some Ramon recipes with much much less sodium. 
He has been better since Thursday.. 

Now that we've caught you up on Alex. 
Here are a few shots of Ry's birthday party. 
A huge huge thanks to Jordan for letting me borrow the decorations. 

We played some French games and some English games. Pass the parcel was a big time favorite. As was this game^... I can't remember what it's called in French but it's similar to Down by the banks.. 
We also had some American French food and had a dance party. 
The kids had green ribbon week at school. The event kicked off with crazy hair day. Even if Gator can't be at the school he still wanted to do his crazy hair too. 
Here is Ryleigh. She loves the idea of crazy hair day. But this Diva doesn't really want crazy hair. 

Gator wants controlled chaos.

The little girls didn't want crazy hair at all...just cute. 
Ry and I ran the Witches Brew HaHa and totally enjoyed it. We didn't even cry. We must be getting better at this. 
Getting ready was half the fun. 
We had an incredible conference weekend and celebrated this little girl. Which deserves its very own post! 

Monday, September 29, 2014

Crazy selfies with the aunties

Apparently because my account is Gator's account, I get all his silly selfies.. And these included the aunties.. I nearly died laughing.. 
For your viewing pleasure...

Hahahaha.. #1 rule is- Don't take life so seriously.. 


Riding in our swagga wagon, picking up some ice cream.. 
Word to my peeps!! 

Wednesday, September 24, 2014

yummy dinner

We still refer to May as the full-contact-eater


but I stayed clean

Ribbon cutting

I think I have mentioned before how grateful I am for all of those people who help our family make amazing memories. Alex was able to help cut the ribbon for the new outpatient building for Primary Children's hospital. I know that some have mentioned that this is a sort hard transition. But here are some very huge positives.... The outpatient building helps separate the sickies from the well kids coming for clinics, everything is new and modern, the jobs it will provide will help our community, the classrooms and meeting rooms are so much bigger and better apt for teaching (which means even better care in the long run), and it is clean and the lunch room looks like a fancy restaurant.... See so many amazing things... That is just skimming the surface of the building and it's awesomeness. Now, I am a creature of habit, so there will be some growing pains associated with going to the new building... but really... most of that is just walking a bit for Gator and he can always ride in a wagon or wheel chair.  
The ribbon cutting event was amazing and the careful planning for just our family was so sweet. We had a special helper the whole time. Thank you Miss Amber. Our kids were the only children at a very grown up event. That had me worried. I didn't want these important people to think I was a bad mom because my babies behavior was a reflection of my parenting skills. But everyone was so amazing. Jeff spoke because we all know how scared of the microphone I am. I wish that I could sound as eloquent and perfect as he does... I wish I could sing as well at the girl who wrote the song and performed it with the music therapy team. She is also a patient at PCH and did an amazing job. 
What was really cool to see was Jacobson construction. Those men and women who worked on making and crafting a building for healing and peace. And because they had huge LEGO guys... And we all know how Gator feels about LEGO!!!
I didn't take many photo's because I was busy managing the small people... 

Thursday, September 18, 2014

MayLee's Dr appointment

MayLee has a pulmonolgy appointment today. Usually it is Alex's cardiology day but they peaked at him on Monday after the ribbon cutting and decided we could skip this Thursday. More details on the ribbon cutting later.. 
May is doing beautiful. She is growing, is as smart as they come, and is so verbal. She has been such a good little NEHI patient. Not really much to report because not much has changed. She will continue to fluctuate in liter flow depending on her needs. She will also stay with her asthma action plan because even though this isn't asthma, there is still so little known that this plan is the best for treating it so far. Our amazing pediatrician will try and advocate for the Synigis injection although we don't know if she can get it again based on her age. I have been on high alert because of the new resparitory illness going around and doc U, reassured me that these precautions were actually wise. So that's it!!! I tried so hard to have her wear a mask while we were at the hospital but she wouldn't have it!!