Skinner Update

Most of you already know about our family and the unique trials we face. But just to make sure we're all on the same page, I want to give a quick overview of what life is like in the Skinner household. I apologize in advance for the length of this post...there's a lot to say!!

Claire and Benson were both born with an extremely rare genetic mutation in their liver that produces an excess amount of oxalate in their bodies. This disease is called Primary Hyperoxaluria Type 1. (PH1) Oxalate is normal in all of our bodies, but our liver only produces a small amount which then gets converted into whatever it needs to be. Their liver doesn't. Oxalate numbers in you and I is around 1.8. Claire's oxalate is around 64. Benson's is at 11.5. So what does excess oxalate do...it destroys.

The kidneys are the first to go because it is their job to filter waste from the body. In that filtering process, it can only handle the overload of oxalate for so long...until they've had enough and eventually fail. Claire has had kidney failure since she was 2 months old and has been on dialysis ever since. Significant oxalate deposits have also been found in her eyes and bones and is inevitably in other organs as well.

Benson's condition has not progressed as fast as Claire's. His kidneys are still functioning normal. No one knows why kidney failure happens when it does...and in fact, we wouldn't have known about Benson condition had Claire's diagnosis not been made. There are no signs of this disease...kidney failure is usually the first sign anyone has to tell them there's a problem. Biopsy of the kidney then shows the kidney saturated in oxalate crystals and it is then linked to PH1. Close monitoring of Benson's kidney and oxalate levels along with high doses of B6 vitamin (thought to help remove oxalate from the body) is his current treatment. Keeping him well hydrated (2 liters a day) is also very important as that helps keeps his kidneys clean and flushed of the oxalate.

Claire's treatment is a different story. She is currently on hemodialysis (done at the Children's Hospital) 6 days a week for 3 hours each day. Sunday truly is our day of rest!! Along with that, we do a different kind of dialysis, called pertineal dialysis, at home. She gets that done every night of the week for 12 hours (while she's sleeping). Ultimately, dialysis 24/7 would be ideal for Claire to remove the oxalate that is constantly being built up in her body. Obviously, that is not possible...so we do as much as we can! Dialysis is the only way, besides the B6 vitamin, to remove oxalate.

The ultimate cure: transplant! For Claire, getting a new kidney or liver alone will not solve the problem. She needs both. A new liver will stop the excessive oxalate production and new kidneys will allow her to be done with dialysis! With Benson, we are hoping we can salvage his kidneys by monitoring his kidney and oxalate levels closely and transplanting his liver before it causes damage. When to transplant his liver is still a big question.

And that's how things go around here!! Many questions are still unanswered but we are simply doing ALL we can to keep our beautiful kids happy and healthy (aren't we all?!!)

With all that said (and much more to come in future posts...remember that was just to catch everyone up to speed with the last 10 months of our lives!) for Clarie's one-year birthday, Sept. 8, I took our camera to the hospital to video what a day in the life of a not-so-typical one-year old was like! Since Jared and I are the only ones who take Claire to hemodialysis each day, I thought I'd give you a little insight to what it's all about. I must warn you, the video is rather long...sorry about that. Just be happy I didn't record the whole 3 hours we are there! Make sure you are sitting around with nothing else to do (ha ha) before you embark on this adventure! Later, I'll posts pictures of the pertineal dialysis we do at home to let you know what that's all about too!

Enjoy!

One BIG disclaimer

I really can't take much credit for the creation of this blog. Thanks to my cousin, Adrian Johnson, for creating the awesome header and background for me! She is AMAZING! I'm so glad she encouraged me to do this! It will be a great way to keep in contact with each of you. Also, Jared and I have decided to make this a private blog--simply meaning that only people who we invite, through e-mail, can read it. So if you have the email addresses of family and friends that I probably don't have, send them to me (nat_skinner@yahoo.com) so I can invite them to see the blog too! Enjoy--and thanks again Adrian!

It's Party Time!!

First things first! Benson and Claire just celebrated their 3rd and 1st birthdays! What a fun week we had! Claire wasn't too sure about the whole cake idea. A combination of the fire on the candle and our singing Happy Birthday brought tears to the sweet girl's eyes! She got spoiled beyond compare with a new fall wardrobe! Benson had a Mickey Mouse birthday party with 6 of his friends...and couldn't have had more fun! We did a cake walk, scavenger hunt, pinata, presents, and of course, CAKE! Here's some pictures to show you what a great time we had!

Happy Birthday Princess

Happy first birthday Baby Claire!!! Yeah for the Princess Heart! I can hardly believe that Claire is already one year old. And what a year it has been! We have been so blessed to have her come to our family. The things we have learned as a result of her being born are priceless. Why? Because they are things of eternal birth. The trials we have had taught us patience, faith, reliance on the Lord, sacrifice, and love beyond what we thought we could experience. Those are the true treasures of life!

Saw a quote on my cousins blog the other day that really touched me. "It is no small thing when they who are so fresh from God love us." What an inspiring thought. Father in Heaven is sending us his love through our children. And the qualities we develop as a result of his love our ultimately what will allow us to return to him as one eternal unit.

What are year! Thanks Claire. Thanks heavenly father.

So how did the birthday girl celebrate? Well at the hospital, of course! And to top it all off, she even got a blood transfusion as the icing on her birthday cake! In spite of all of that, she had a wonderful birthday. She wasn't too certain about the whole "play in the cake idea." In fact, the fire on the birthday cupcake or our lovely happy birthday singing or a combination of the two - about brought this girl to tears! I think playing with the balloons was her favorite part of the day. Not much better than a crib full of fun balloons! She got spoiled beyond compare with a brand new line of dresses and clothes for the fall!  She is most certainly one of the nicest dressed 1-year old girls in town!  

Labs not Good - Adjustments

 Where is doing well. But unfortunately her labs don't reflect it. We took an oxalate two weeks after her being on hemodialysis and the level actually went up to 80! It was 76 before that! A surprise and disappointment to all of us. So we're going to make a few changes. We're going to start giving her medicine after her dialysis instead of before so that the meds we give her won't be dialyzed (taken) off by the dialysis and basically not do her any good. And then we are also going to add Saturday to her dialysis schedule so she's not going 2 days in a row without dialysis. So for now, will still have Thursdays and Sundays off! Today we also drew two more oxalate blood levels – one before dialysis so we can see where she's starting at and one after dialysis so we can see how much is coming after the session. We are hoping there's a big difference between the before and after levels!

We're getting ready to celebrate both hers and Benson's birthday next week! Claire is on the 8th and Benson is on the 14th. It will be fun, but a busy week! My mom is coming in for the week to help out and to help me throw a small Mickey Mouse party for Benson. Nothing big, just something to let him celebrate his special day! Benson is doing well so far with going to his friends houses while I am gone with Claire. He's been a trooper and it's really quite understanding of all that we have to do for Claire. His spirit was definitely specifically prepared to come to our family and deal with the challenges we face. He already knows something about sacrifice, patience, and love that I don't expect most toddlers understand yet. 

I am doing well. Still trying to sort out what I can get done when and where. It is a tricky balance we all face - getting stuff done and spending quality time with our kids. It all seems to work out somehow though! What really helps me get through all of this is remembering that this is just a season of my life. It won't last forever! (Although sometimes it seems like it may.)  It is just another chapter in the book. And I can and will enjoy this chapter! 

Back to the Hospital

Unfortunately we had a little sickness going around these past few weeks. Benson started out with it – a fever and a cough. The cough lingered for a bit, but he seemed to recover quickly. Thursday was Claire's day off from dialysis but unfortunately she didn't have a good day. She was starting to get what Benson had and just didn't feel well. On Friday during her dialysis she spiked a temperature to 38.5°C and that makes them suspect a possible infection in her hemodialysis catheter line.  It is routine that if they spiked a temp greater than 38.2, they draw a culture and start antibiotics in case she does have an infection in her line. 

Along with that routine procedure comes in overnight stay in the hospital. So back up on the eighth floor we went. Room 802-our six week home away from home-was taken so we got put in room 817!  Kree and Amie were our nurses and we got to see many other nurses there we had known before. It was kind of fun to see so many familiar friendly faces and get exceptional care by our good friends.  But still not that fun to be back in the hospital.  Being in the hospital it's just not on my top picks of places to stay overnight! Benson and Jared came out for dinner and we got to go on a little walk around the hospital. The worst part has got to be the lack of sleep we both get while we're there. It's just hard to get any good sleep with vitals every four hours and doctors and nurses walking in at random times just wanting to take a listen to her heart! OK I don't need to rant and rave too much, I'm just grateful it was just for one night! I don't know how I did it for six weeks before! I guess we were just blessed with what we need when we need it. Endurance! We got to come home Saturday afternoon and get a great nights rest that night! Claire is doing much better now and turns out she didn't have an infection in her line, just the fever Benson had passed on to her. That's a blessing. 

Sacrifice

It has been two weeks now since we began our new journey. I first have to comment on how mature my kids are. Little Benson, not even two years old yet, know something about sacrifice. He knows he has to sacrifice for his little sister. Today was the first day I actually had to drop him off at a friends house from 7 AM to 1 PM while I took Claire to the hospital. I bent down when we got to Lance Christensen's house and told him once again that I had to take Claire to the hospital to do her dialysis and he was going to play with Lance while I was gone.  I reassured him that I would be back to pick him up after Claire was done. Just about when he's eating lunch. And the look on his his face right then was one of sacrifice. Not that he was being punished by playing with Lance and having a great time at his house all day. But I assure you that given the two options, he would have chosen to stay in the comfort of his home and play with his mommy and baby Claire instead. He knew he had to do this for mommy and Claire. He didn't put up a fuss about it. He didn't even take a step back towards the car. He just said "OK mommy" and walked into Lance's house with a look of seriousness and courage.  What a sweet boy!

And sweet baby Claire-not even one year old yet. She knows a little something about sacrifice too. As one of her nurses has said, "she is so mature in the way she handles being on dialysis."  Although Claire can't talk yet, her face, her mood, and her expressions can say much more than any words. Now that she is familiar with the nurses who work on her, she is tolerant and even excepting of what they have to do to her. One part in particular where this is evident is when they have to change her dressing on her catheter site. She sits there, still as a rock, keeps her head straight forward, and looks at the nurse changing the dressing out of the corner of her eyes. She doesn't move a muscle. It doesn't feel good to have the bandages and tape removed from her skin. But she doesn't whimper, she doesn't wiggle. She lets them do what they need to do. She watches them every step of the way. She trust them. 

And in these small, simple examples, I suppose I can get a glimpse why Heavenly Father allows hard things to happen to good people.  We all have to learn to become like him.  We do that here in mortality through experiences that refine us and teach us a little something about, in this case, sacrifice!