We Are Facing Some Decisions

As of right now, endocrinology is working on getting us the IGF-1 injections.  We found out they are 2x a day injections, must be refrigerated, and have to be administered either 20 minutes before or after a meal.  We actually just got a letter from our pharmaceutical insurance stating that they received notification that we were to start getting the injections, but are waiting on some more verifications. 

However, we aren't sure we are going to go that route.  We had a very big appointment with GI today, and it looks like we are facing some big decisions.  GI's main concern with not "fixing" Macy's liver is the long-term effect it can have on her cognitively, and not so much worried about growth.  When your blood is not getting "cleaned" by traveling through your liver, it can cause your ammonia levels to rise, and cause some cognitive problems.  Ammonia levels should normally be less than 35.  Macy lives in the 90s and 100s.  This is very high, and not safe long-term.  So, Dr. Molleston feels it is time to intervene.  This is something I had a feeling was coming considering the long-term effects it can have for her brain, her lungs, and her growth.  It's just very hard to hear and think about. 

Dr. Marshalleck, is our Interventional Radiologist at Riley who performed a venogram on Macy in 2008.  Dr. Molleston, our GI said we could have him perform another follow-up venogram.  He would additionally do a ballooning, while in there,  to see if we could close the shunt and other veins in her liver would be able to receive the blood flow so that it would go into her liver properly.  It is a tricky procedure that measures pressures and has quite a bit to look at.  If it appears that her shunt can be closed, then we would most likely travel to Dr. Superina in Chicago (one of the only doctors that currently performs these repairs) to have him close her shunting in a 2-3 step banding procedure. 

So as of right now, Dr. Molleston has a call in to Dr. Superina to get his professional opinion about Macy's case.  She also had communications open between a few of our other specialists to touch base, and also get their opinions.  So if we go the "fix" route, and it works, Macy will not need the IGF-1 injections.   I don't know of the plan right now, if the "fix" turns out not to be an option.  I am not going to think about that....we are thinking positively! 

This was so much for Jeremy and me to take in this morning.  I kind of feel like I was in a fog the rest of the day.  It helped to talk with my mom and a friend at work today.  It helps me think things over when I blog as well.  Nevertheless, Jeremy and I have some big decisions to make.  Do we want the procedure for Macy?  Do we want to do part of it at Riley and part in Chicago?  Do we want to do it all in Chicago?  We also have lots of questions.  What are the main risks of the procedure?  Is it reversible if problems arise down the road?  Do we have to have a follow-up revision years down the road?  Is she cured?  I'm not going to lie, I'm worried.  I am worried that we may make the wrong decision.  I am worried that we may mess with something and cause more problems.  I worried what might happen if we do not "fix" this.  I am worried mostly about Macy....how will this affect her?  We will keep posting as more information comes.  Thanks for following our family blog and for all the prayers! 

5 Fantastic Places

 

What are your top 5 places, within reasonable driving distance, that you want to take your children?

It just so happens I have 5 places!  These are places that we would most likely never fly to, and can easily drive.  Considering that Macy will be 10 in January, I have set a reasonable goal to take the girls to all of these places before Macy graduates high school. 

1.  Mackinac Island- I went here when I was in elementary school and absolutely loved it!!!  The old town, the scenery, the carriages with horses, the ferry boats....it was all so neat, and I know my girls would just love it.

2.  Mammoth Caves- Located in Kentucky makes this a very quick and easy place for us to travel.  I loved going to the caves when I was little.  It cool, creepy, and awesome all mixed into one.  Macy is very cautious about most things, especially things that are deep, dark, and secluded.  I know this would be a big challenge for her, but I would love to watch her face this fear!

3.  Washington D.C.- I was in 5th grade when I went here, and thought it was very neat.  It is such an educational place, that brings so much American pride into your life. 

4.  Branson, Missouri- Neither Jeremy or I have ever been, but we have heard such wonderful things about Branson.  We hear it is a fantastic family place, and that is right up our alley!

5.  Gulf Shores- I went here as a young child with my family.  My grandma Bettie and I spent hours collecting shells in the ocean.  It is still such a wonderful memory! 

My Health Choices: What Works for Me

I am by no means a fitness expert.  I am not super fit, or skinny, or have an awesome body....boy do I wish I did, lol.  I am, however, a person who does care about my health.  When Jeremy and I first married we ran, hiked, rode bikes, and roller bladed together all the time.  We had only healthy food in our pantry and fridge.  It was just how we were!  Jeremy's sister often tells about the time when she was in high school and she stayed at our house while we were on vacation.  The closest thing she could find to a "snack" was yogurt.  She couldn't believe we didn't have anything sugary in our house. 

I got so sick during my pregnancy with Macy, and then had preeclampsia and was not able to work out.  So that part of my health was fading.  Then my healthy eating started fading as well.  After Macy was born, I was just trying to make it through the day.  During hospital stays we started finding comfort in sneaking down to McDonalds on the first floor at Riley, and getting milk and cookies.  We found comfort in running down in the morning to grab a hot chocolate.  Once home from hospital stays, there were many days that I came home from working full-time, had marathon feedings with Macy just to get her to take 3 oz., cleaned up her vomits 3 plus times,  suctioned her trach  20 times in one evening all before putting her down for bed.  Knowing that I had to suction her again in the middle of the night, re-attach her leads from her monitors when they came off over night, and refill the water in her humidity machine at least once in the night, allowed me to think I deserved any snack I wanted!  This may be an excuse, but to this day I do think I deserved some kind of relief.  Relying on food probably wasn't my best choice, but at the time I had no other outlet.  After doing that for so long, you start to get used to eating that way....or allowing yourself to think you deserve that unhealthy snack,

I am not going to lie that I have struggled throughout the years with going through phases where I don't quite eat how I should or exercise like I should.  I'm human!  I have really been working on eating much better for a couple of years now, but exercise wasn't always a part.  In February, Jeremy and I decided it was a must!  We've been working out 6-7 days a week since.  It is hard, it is time consuming, and there are times that I don't feel like it.  Knowing that I have a workout partner and someone to hold me accountable has been such a critical piece to all of this.  Jeremy and I have been supporting each other on a daily basis.  We do our cardio workouts separate, and our strength training together. 

Here is what works for us:

We've done P90x, and still have the DVDs, so sometimes we use them for some of our workouts.  My mom also introduced us to Tabata style workouts and Fit Crew style workouts.  We do these as well.

Sunday: Choose from either a Core workout, Kenpo workout, or full body Tabata workout.
Monday: Cardio
Tuesday: Lower body strength training
Wednesday: Cardio
Thursday: Upper body strength training
Friday: Cardio
Saturday: Day off, or long walk

We try the best we can to stick to this schedule, but there are just some days that we have to switch things around.  We've had a lot of fun working out at the lake this summer.  We use the hill to do hill climbs and we kayak across the lake.  It's been a lot of fun to take things around us and turn them into a part of fitness.

One of the best parts about being physically active is that our girls often join in on our workouts.  They are so cute trying to do push ups, lunges, and weight lifting.  I want my girls to know that people don't just workout to look good, they workout as part of their total health!  I want it to be a part of their lives!

A Little Medical Update!

No pics this time, just a little medical update about Macy. 

Macy has been diagnosed with Severe Primary IGF-1 Deficiency.  This is different than a Growth Hormone Deficiency. This is a rare condition....1 in 10,000 to be more precise.  A person with Severe Primary IGF-1 Deficiency is smaller than 99% of their peers, has had a formal test for growth hormone deficiency and is producing an accurate amount, yet has an IGF-1 deficiency.  IGF-1 is Insulin-like Growth Hormone Factors.  We believe this deficiency is due to her liver malformation. 

Our endocrinologist found the supplement that they would like to start giving to Macy.  It is called Increlex, and is a 2 time a day shot.  Fortunately we will be trained by a nurse at our house so that we can prepare and give the shots at home daily.  We have started the process of getting this medicine.  This referral process can take months to get going.  The first step was for our doctor to fill out and send the application and paperwork for medical necessity.  This goes to the company, then through insurance, then back to the company, and so on a so forth.  Insurance could deny coverage, so prayers for coverage would help! 

We will still be visiting our GI doctor later this month to discuss her liver and if or what we need to do next.  Our endocrinologist is also discussing this new med with Dr. Hainline (our metabolism doctor), due to some side effects.  The main side effect is hypoglycemia (low blood sugar).  Well, Macy is already hypoglycemic, so we can not allow room for an additional drop in blood sugar, so we will need to tread lightly with this.  We will need a plan put in place, and we will need to monitor her blood sugar carefully.  At this point, I have no idea if this will be something we will have to do at school, or how often it will need to take place.  We will just wait for word from the doctors and go from there. 

This is very difficult for me for many reasons.  1.  I am not a patient person!  2.  I am a very detailed person when it comes to Macy's medical care.  3.  I worry myself sick about medical unknowns (literally sick).  4.  I am simply a mother, and just want the best for my child. 

Macy knows we are trying to find answers, but Jeremy and I have decided not to tell her anything until we know for sure and have a plan.  She has a lot of anxiety, therefore there is no reason to provoke it.  We will keep you updated as our questions are answered.  Thanks for continuing to follow us, and for praying for our family (particularly our sweet Macy).