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".

So far this year.....in pictures.

Exercising

She found Mema and Papa's bag of travel snacks including a can of mixed nuts.  And it confirmed she has an allergy to at least one of them. 

 Coolness.

 Everyone wants to help.

 We installed gates on the kitchen so this doesn't happen any more.

 We had an ice storm and "went sledding".

 They love each other.  They played outside all day in the ice. 

 Shiloh "wanted some special time with Aggie", since they share a room; after we put them to bed we really don't know what goes on.  I hear over the monitor "Hi, Shiloh....Hi, Shiloh.....Hi, Shiloh.....(repeat over and over)" After several minuets of this greeting, I checked on them and found this.  Shiloh stayed in her crib for about 45 minuets with Aggie saying "Hi, Shiloh"an in-numerable amount of times. I think she liked "special time with Shiloh".  Shiloh is so sweet and patient with her. She loves her sister.

 Bath time help.

2nd Annual Valentines tradition, family fondu night.

 Salem's getting big!

 She's so cute.

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.

The New Job

Mike has started working for TKI. It is a company, contracted by a company, that is contracted by the government to teach foreign students how to fly. This class is in a classroom. It's lectures for the students, prior to going to the squadron. He dresses up all fancy in civilian cloths with a tie and such and shares his knowledge of pilot skills with Saudi Arabia gents. This is his 3rd week with TKI and so far he's enjoying it. It's 40 hours a week. He likes his fellow instructors. And he is still flying 5-7 days a month with the reserves. So needless to say, it's been busy around here. I am thankful for the Lord's provision.

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.