Fall 2014

She made it to 4!!!

Aggie celebrates four years of life today!! Statistically speaking this is good news. We were told after receiving her diagnosis that if she makes it to four years old, she is much more likely to live to adult hood.  Of course none of us really know the number of our days, the bible says. And since we were all diagnosed with a genetic mutation of the heart at birth (aka. sin), we are all terminal! But in Christ's kindness he came and lived a perfect life as a man, died the brutal death on our behalf, and over came death, and is now seated at the right hand of the Father praying for us perfectly. He made a way out of our desperate state!! Thank God!!
I am very thankful to know that while I still walk on this earth, before I am with my savior in heaven where I so long to be, I will get to experience the life of our sweet Aggie Rae a few days longer. Her smile, her smell, her velvet soft skin, her beautiful blue eyes and feather soft hair and her corky sense of humor. Her joy is contagious, a beaming Ray which is her namesake. If not for you sweet one, I would not have experienced some of my greatest joys and deepest sorrows, that have shown me the the unending, unchanging consistency of my Fathers love.
Happy 4th Birthday to my little monkey, stinker-bug, turkey-lerkey, Aggie-doodle, Aggie Rae!

A friend shared this beautiful song recently, I thought it was timely. I'm mostly posing it because I don't want to forget it, but you might enjoy it too.

I thought I picture of her with my mom was appropriate. I feel certain that she is her favorite person. Every time my phone rings she says, "Hi MeMa, Hi MeMa!" over and over.  And if my mom happens to be on the other end she say's hi back over and over again. She has to have said "Hi" to Aggie thousands of times by now. If it's not my mom, I must go to another room so that the person I'm speaking to and myself can hear over all the "Hi Mema's".

Walking!!!

My heart is full as I write the the title for this post.  Full of thankfulness and a little bit of disbelief. In the sense of "Oh my goodness, I can't believe it!.  Can you?"
The story in full, well.... as full as it's going to get.
A few weeks ago both Aggie's teacher and I pointed out that Aggie doesn't really need our hand for walking.  She could have a loose grip on our little finger and be walking along side us fine. But the moment the little finger is removed, down she would go. It was a learned behavior, a sequence of events; finger removed, I must sit. So at her IEP meeting two weeks ago we all (teacher, therapists, myself) discussed what we could do to break this learned behavior. What we came up with is not what actually got Aggie to walk. The point to this is, we saw the ability.
The story as told by the teacher and OT.
Last Friday, April 4th, 2014. Aggie's OT Dawn, entered the class room for therapy. Aggie was excited to see her. The teacher, Donna, was walking her toward Dawn and let go of the hand a few steps prior to her getting to Dawn and Aggie took a few steps on her own to finish the distance. They all noticed.  So Dawn left the room and re-entered to re-inact the scene but Donna released Aggie's hand a little further away from Dawn and Aggie again completed the walk on her own. Repeat this scene a few times. (They are so thoughtful and good to her). I'm not sure what all happened in between, but I'm guessing by all the video they showed me, that they went to get the PT, Neoly. Because most of the videos they have shown me, are of Neoly walking behind Aggie and singing, and Dawn taking video and shaking keys. They told me they had her walking the halls of the school most of the morning. Practice makes perfect.! The initial video I posted on Facebook is the only one they could send to me on my phone b/c it was the shortest and smallest file. And it was of her walking up a ramp!  This video Dawn took the time to email to me and it is a bit longer. When I arrived to pick Aggie up from school they had her waiting outside with OT, PT and conductive PE teacher, and had her walk to me. How  special and thoughtful. I cried... I think I even snorted... What can you do? She's walking.
What's next for Aggie?
Though Aggie is taking independent steps, she still needs to learn to get herself into a walking position to begin walking. We currently have to stand her up and say "go get (toy/person of choice)". And though she can pull her-self to standing, she does not know how or feel comfortable transitioning to walking from this position, which you can see at the end of this video once she finds the wall. Also endurance and strength. These are the things I'll be praying about and we will be practicing. Most of Aggie's physical developments are skills that we've had to teach her, one step at a time, so we will continue in this fashion. I am thankful that she is still able to learn!

Amendment: Regarding Lab Work

 I received a call from Aggie's genetics dietitian yesterday. On behalf of the doctor. Baylor University has made an amendment to one of her labs drawn in 2012. They have found another mutation. The same mutation was found in an infant baby girl who died shortly after birth. Therefore they are more concerned of the progressive nature of our circumstance. It was all read to me over the phone, it will be mailed soon. There is no name other than the specific gene numbers and letters. It does not change her diagnosis of PDCD or treatment. It basically doesn't change anything other than potential for quicker progression. We already know Aggie has a progressive disease, and will not live a "normal" life. So we will keep on living and loving her. It did cause the doctor to express further concern about the other girls being tested (being genetic in nature and X linked), which has been denied by our insurance.  We have appealed twice with no luck. Perhaps the new information may help it to go though? The Genetics doctor wants to see us again. Not urgently. So we will plan a trip to Birmingham soon. I always hate spreading news like this, but I know everyone likes to know. 

On an encouraging note I just want share that some of the biggest things God has taught me though all of this. 1.He hears our prayers. When I was pregnant with Aggie God laid it on my heart that she was not going to be "normal". He cared so much for me that he prepared my heart early. I started praying for her then that he would have mercy on her, heal her and sustain her life. That Aggie is alive today and not with the Lord, like the other child with her mutation attests to His hearing and answering those early prayers for her. 2. He cares much more about our holiness than our comfort. It is very uncomfortable at times walking this road He has laid our for me, but it has been a huge source of sanctification and making me into Christ's likeness. 3. He cares more for my child than I do. He created her. I have laid her at His feet many times for surgeries and tests, in-trusting her to His care. Her creator can provide for her in innumerable ways that I can not. 4. It has caused me to face fears that I thought might kill me. And remove my children as idols so that I might see my savior more clearly. For that I am thankful.

Mr. Schloss' Job and Ketogenic Diet

After speaking with a close friend this week who lives far away, and is kind enough to read my blog I realized that I have not put any updates of some major life events in our home.  Mostly this is due to time constraints, and my attempt to balance something that is very unbalanced.  I try not to spend much time on the computer. We have a house full of people here at Schloss Casa that require much of my attention.

On to it then: Mr. Schloss's Job or should I say Lieutenant Schloss' Job.
Mike is now a Lieutenant in the Navy Reserves.  He separated from the Marine Corp this past summer with the intent of doing an inter-service transfer to the Navy reserves.  I am so thankful to the Lord for the time we had to plan and prepare for this transition as we knew it would mean at least 40 days of unemployment.  And in typical military fashion, it took way longer than expected.  Mike was unemployed for 6 months. He started checking in with the Navy in December.  For the first couple months he has been able to secure "active duty" status with the reserves, which is a blessing finically for us as he considers and looks for jobs he can do along side or with-in the reserves. Thank you for your prayers!

On to other big news....... we paid off or mortgage!!! So this reduces our monthly bills significantly. Pretty exciting!

Aggie has been on the Ketogenic diet for 6 months now.  It is the only treatment that is being tested for her diagnosis.  Her diagnosis is Pyruvate Dehydrogenase Complex Deficiency (PCDC). It is a metabolic disorder and mitochondrial disease. Her body is unable to use carbohydrates for energy. Instead of making energy it would make lactic acid, which would accumulate in her blood and cause damage to her brain.  Which is why her brain is already abnormal. The diet is, in theory, suppose to slow this process down. It trains her body to use fat for energy instead of carbs.  I have been thankful to see the diet has benefited her in some great ways in this short time; proper weight gain, increased energy, are two big ones.  She still gets crabby, and can be difficult to manage, but the incidences have decreased. She naps less because she has more energy.  Her main source of nutrition is a formula called Keto-cal designed specifically for children on this diet. It tastes like melted ice cream and Salem would be thrilled if it was what we put in her cup, as we often find her confiscating Aggie's cup. I do continue to make and weight out grams of food for recipes and meals for Aggie. We are in more of a groove now with that and have found some of Aggie's favorite things to eat.  One thing that has been disappointing for me is Aggie's lack of desire to eat. She does not eat much or often. I have had to relinquish my desire for her to be "normal" in this area as well, as it may never be part of her life. Eating is more a form of entertainment for her and her ability to consume enough to meet her nutritional needs is not there.  She loves to eat ice chips, which she is allowed in unlimited proportions. She requests it often.  One negative side effect of the diet is constipation, and we have worked out some kinks with that. We now use MCT oil as part of her evening meal to keep her regular, which works most of the time. Occasionally medicinal intervention is necessary. One other large risk of the diet is kidney stones. We have no reason to think she has those and have been running water through her feeding tube during nap time in an attempt to prevent them.  We still run the feeding pump at night most nights which generally complets it's feeding at 2:00 a.m. and I tend to it. She is suppose to drink 20 ounces of formula in a 24 hour period, so what she doesn't drink in the day she gets at night. All in all we are seeing that the diet is affecting her positively and plan to stick with it as long as it suits us.  It is some extra work for both Mike and I. Mike compounds(crushes) all her supplements that she receives twice daily, so that they are carb free and can be given with liquid though her tube. Any additional medicine she needs I crush and weigh on a milligram scale so it is an accurate dose for her weight.  Thankfully this is only occasionally that she has need for this.  The diet has been good exercise for my brain.  I have been re-acquinted with some intense algebra, could probably eyeball what 15 grams of butter or banana is, and have tried my hand a pharmaceuticals.  We are thankful for God's grace to do what He as called us to in this difficult season of life.

November 2013 Photo Summery

Thanksgiving and Birthday Friends watching Charlie Brown.

 Lost his 1st and 2nd tooth.

 A strong fortruss to shoot down with daddy

 A meal with the Todarov's from Bulgaria.

  A little doll under the tree.

 Rocking

 Resting

 Helping

 Adventure

(She got stuck like this)

Birthday Fun 

 Morning birthday suprise

 Self-made knight in shining armor

Mercy-a generous gift

I was driving Aggie to school this morning. She was in the back seat saying.... ready, set, go! Over and over and over. She "get's stuck" a lot. We joke that it's like an old record with a scratch.  It often makes us all laugh, and guess what...?? She keeps doing it. :) But this morning it was different, it was making me smile, because it reminded me of the silly games we play at home and allow her to determine when they start. It's one little way that she's included in our tomfoolery. But also I was reminded of a different morning in Aggie's life, where the weather was cool like toady. I looked up the date; February of 2011.  It was the morning we got the results of her MRI.  I remember aspects of it so vividly.  We saw a picture of her brain, it caused me to feel frozen. A later doctor likened it to a raisin instead of a grape. Even the untrained eye can see such a significant malformation. Not only was the size smaller, but there were 2 large holes in the middle as well. We all have ventricles in our brain, (holes) the problem was not with the space it's self, but rather the size. Anyway, Mike and I quietly listened to the doctor as he explained the picture. We maintained self-control and asked objective questions as if in a business meeting. We learned generally that these malformations would likely affect Aggies motor skills and communication.  So Mike and I took Aggie out to the van and got in, I looked and him and said, "this is pretty sad, right?" Finally... I could cry.  His eyes were welling up, "yes" he replied.  Our little baby... who would she grow to be?  How will this affect her?  Now..... fast forward a couple years. "Ready...set....go!"
God has been so merciful to us!!! I am amazed at the progress Aggie has made. She is a chatter box! Loves to talk about everything. We don't always understand her, or it may be the same thing over and over, but she can let us know if she wants something or does not want something. This is communication! I will forever be grateful for the sense of humor part of her brain; because it's on full throttle! She knows how to be funny and knows when we are being funny. And her motor skills are still progressing, she can crawl F..A..S..T! We call it her speed crawl. She has a turbo boost of sorts.  And here recently she has been showing good progress with assisted walking. There was a day that we didn't know if Aggie would sit up.  When I picked her up from school today she walked down the hall and out of the building holding my hand. Praise God!!! What a sweet thing it is.

August 2013

Salem is getting so big, she is now 8 months old and has become proficient at sitting up.

 Cheeks, just love em!

 Smiles

 Beautiful Baby

 I was trying to take Salem's picture sitting up and Aggie kept getting in front of me saying "Cheezzz".

 Shiloh was coordinating her outfit with the fan Mema and Papa got her from Spain.

 I asked the kids to go cheer Aggie up, I was cooking.  This is what they were doing, Hosea was hoping on his ball, Aggie loves this and Shiloh was letting her hop on herself.  Sweet little lady. And it worked. I find this scene often, but in different configurations, it almost always involves someone hoping, jumping or bouncing.

 We have accomplished 2 weeks of school!  Hosea is officially a kindergartener and Shiloh 2nd grader. So far we are all enjoying our new schedule, and chipping away at some great goals along the way.

 This is what our kitchen table looked like one day. I can't say it is a rare occurrence. It really captures this season of life for us. Note the portable Dvd player to distract Aggie while we attempt to get her to eat, the iPad, my laptop, mike's bible, Aggie's Mic-key connector, Salem's puffs, bill binder, random cup, 2 kids smoothies, and 2 baby trays (yellow and white). I think i see Shiloh's schedule cards too(no wonder she can never find them).  It is amazing we manage to eat 3 meals a day at this exact same location, I'm guessing this was a Sunday evening b/c of the smoothies? But may be not?

Fort Pickens Photo Shoo

Early in the summer we took the kids out to Fort Pickens to get some shots of them.  We got some nice ones, of course things hardly ever go as my idealistic self would prefer, but all in all, I'm pleased.  I've even made a photo card to send out to friends and family. I have them printed sitting in a pile somewhere. :/

I got the most good ones of Hosea, I guess he was feeling most cooperative.

 Best one of them all together.

 That lil nut of ours.

 She's getting hard to photograph, she's mastered all the poses and fake smiles.  But she has such a beautiful real smile, I just want the real smile. 

 This was an action shot that turned out.

 What a little doll, love her!

 I feel like this captures a lot of our family dynamics, uncensored. 

 Bright eyes.

Diet Up date

Hey Friends,
If you think of it pray for us. The diet isn't going the best. It is causing some serious constipation, and thus causing Aggie to not want to eat. So all this food weighed to the precise gram in a perfect ratio of 1 gram carb/protein to 4 grams of fat, and calculated to the exact calorie amount is not being consumed. :(

I wake up in the morning to cross multiplication of fractions in order to make scrambled eggs and bacon. The recipe looks like this:

11.8 grams of 36%cream
7 grams of "10% fruit" (we've been using orange and strawberry)
4.6 grams of bacon cooked crisp (oscar meyer)
10.1 grams raw egg mixed well
3 grams MCT oil
7.3 grams canolia

Then do your best to mix it all up and make it look delicious so a 3 year old who acts like a one year old so she might consider swallowing it instead of throwing it onto your pantry door and floor.

Incase you aren't familiar to how much a gram is, off the top of your head, b/c I had no tangible comparison until this past week, this recipe makes up a meal that could fit in the palm of my hand. It equals 175 calories, I know this b/c I did the math to ensure it. Basically 2 adult bites of egg/bacon combo and 1 bite of fruit. On a positive note, if she's not eating I don't have to cook. :/

Additionally Our Hosea has an ear infection, he is a little pitiful. We needed to reorganize a couple rooms in the house to Aggie proof them, that's mid task.  And our back yard has 3 trees laying in it that were cut down 2 days ago.  All the while trying to provide somewhat nutritional meals and do the maintenance cleaning required to sustain lives and run the home.

I'm carrying these verses around in my pocket;
Isaiah 43
"But now, this is what the Lord says- he who created you, O jacob, he who formed you, O Israel:  "Fear not, for I have redeemed you; I have summoned you by name; you are mine.  When you pass through the waters, I will be with you; and when you pass through the rivers, they will not sweep over you.  When you walk through the fire, you will not be burned; the flames will not set you ablaze.  for I am the Lord, your god, the Holy one of Isreal, your Savior; I give Egypt for your ransom, Cush and Seba in your stead.  Since you are precious and honored in my sight, and because I love you.  I will give men in exchange for you, and people in exchange for your life. Do not be afraid, for I am with you."

 Amen

Quick brain unload

 We were on a month long road trip June 18th to July 14th ish. We went to MO to see my family then to OH to see Mike's then up to Cleveland where we were able to speak with a Doctor who had recently done research on children with PDCD and how the ketogenic diet affect them. The diet has had nothing but positive effects on these children according to the study. The Dr. spent an hour and a half answering our questions, it was so helpful!! We also learned some good news.
1. Aggie looks very well for a female with the disease. That she has any form of mobility at all is remarkable. We are so thankful, and hopeful that she may someday walk.
2. If a female with the disease lives to be 4 years of age she will likely live into adulthood.  Since Aggie is 3 and well, things are looking good for a longer life. Good news to us.

Mike is out of the Marine Corp, he has applied to the Navy Reserves and once he is in; he is hoping to apply for a full time job with in the reserves. He is currently unemployed and the paper work for this transition can take 1-6 months.  He's getting a lot done around the house now that we are home.

We went to a parent practicum for classical conversations, our home-school group, for 3 days in July.  I did the tutor training, as I'm going to be a "tutor" (aka teacher) in one of our children's classrooms when we all meet on Tuesdays.

We started Aggie on the Ketogenic Diet 4 days ago. She is officially in ketosis, which is the goal.  It is suppose to supply a more useable form of energy for her.  She won't eat much(which is not the goal), but is drinking her new formula well.  Her temperament is relatively the same, which has large fluctuations on a normal day, so it's really hard to tell how she's feeling.

Salem is starting to roll all over the floor to get to what she wants. She is nearly 7 months old. It's nice to see her progressing. She's pretty cute and sweet. We love her!

Salem getting cup

Things like this tickle me.  I think it's b/c most of the things Aggie has done/accomplished have been taught by much repetition. So I have forgotten how healthy little ones are naturally curious and try their luck with learning all on their own.

Salem is a prodigy, she has grabbed a cup :)

On this note: post Aggie parenting........ I still, to this day, every time I change Salem's diaper/cloths or even just hold her, I find my self being cautious around her tummy as if to be sure not to disturb her G-button.

Aggie one hand walking

We are getting closer!!!

Genetics-Pyruvate Dehydrogenase Complex

The Genetics Doctor called today.  The results of Aggies muscle biopsy are back.  The muscle was tested twice. One test showed 7% muscle function and the other showed 40% muscle function. 50% is normal. The reason for the variation is that PDC is an X carried gene mutation.  Girls have two X's per gene.  So if one X is 7% then the other could be pulling the weight. Based on Aggies "clinical findings" (small head, brain damage,ect) it is evident that the brain is affected by the mutation.  If Aggie were male she would likely no longer be with us.  Since she is female, prognosis varies, but rarely exceeds 20 years of age.  The treatment is a ketogenic diet. We will go to Birmingham next Wednesday to learn about it.  The diet is suppose to prevent further damage to the cells by primarily using fat for energy instead of carbohydrates.

This is a portion of wikipedia's definition that explains it well.

"Pyruvate dehydrogenase (PDH) deficiency is a congenital degenerative metabolic disease resulting from a mutation of the pyruvate dehydrogenase complex (PDC) located on the X chromosome. Although defects have been identified in all 3 enzymes of the complex, the E1-α subunit is predominantly the culprit. Malfunction of the citric acid cycle due to PDH deficiency deprives the body of energy and leads to an abnormal buildup of lactate."

Hello

Buddies

We may have some noise in our future :/



Hosea loves to play with Aggie.  Sometimes when she is napping he will be looking for her to play and will ask if she is napping, and be disappointed when he finds out she is. He is a sweet big brother to a special little girl.

Aggie's Field Day

She took a little stroll in the gait trainer. She's not really a fan, but they keep trying.

Also amusing....

When Aggie is done eating she is in the habit of taking her bib off over her head and throwing it on the floor. This particular day I had left her in the chair a bit longer than she would have preferred.  She tried to take her shirt over her head and succeeded, but she didn't have the arm thing figured out so well.  Small things like this make me chuckle.

Funny

Hip Dysplasia

Aggie's pelvic/hip x-ray revealed that she has hip dysplasia.  We will need to see an orthopedist for this.