Hello friends!  Hope your Thanksgiving was full of family, fun, and festivities.  We have had a very nice couple of days off work and school and have very much enjoyed the family-time. 

As you may recall, Rees was recently diagnosed with von Willebrands disease, a blood disorder that affects the clotting of his blood.  Fortunately, the disorder does not seem to negatively affect his normal day-to-day activities.  Though, he does now have recommended restrictions as to the activities he should and should not participate in.  He shouldn't pursue a career in football, wrestling, soccer, ... high contact sports and similar activities.  Because of his craniofacial issues, we had planned to steer him away from those activities anyway.  The only reason we realized there was likely a problem with his blood is because of the number and extensiveness of the surgeries that Rees has had.  So, in one sense, we are fortunate that Rees has this type of blood disorder, if he has to have one, I suppose.  The plan is that when Rees has a surgery that he would receive an injection of a medicine that induces the release of von Willebrand factor (of which Rees is deficient) from its storage sites in endothelial cells.  I don't completely understand it but basically with the medicine in his system, Rees should clot faster, preventing him to have excessive blood loss as has happened so many times before.  

If you remember, Rees had to go to the hospital for a trial injection of the medicine to determine if it will actually work for him.  This medicine is commonly given via a nasal spray, however, due to Rees' facial anatomy, we opted for confidence and had the medicine injected intravenously.  

So, here's the deal ... without medication, Rees' von Willebrand factor is at 30%.  At the first blood draw, his factor was at 68%.  That IS a significant increase, however, it really should be near 100% for craniofacial surgeries.  After an hour, his level dropped to 54%, which shows that Rees' body cleared some of the medication.  The way I understand it, these results are not optimal; Rees did not respond to the medication as well as was hoped.  This will mean that other measures need to be considered for Rees' future surgeries.  At this point, we don't know what those measures might be.

The other thing that recently happened is that we were referred by a very reliable and knowledgeable source to have a consultation with a plastic surgeon in Chicago, one who is very well-known and highly regarded for complex nasal reconstructions.  The appointment was very last-minute but since the kids were off school for the Thanksgiving break, we decided to try to make a day of it, instead of leaving them home.  

We had a great visit in busy-bustling-blustery Chicago!!  

I don't know what this giant-silver-jelly-bean-looking thing is ... BUT ISN'T IT something?  It's near Millennium Park and is quite the attraction. 

This is the view from underneath it, looking up.  Wow!!

There is definitely SO MUCH to see and do in Chicago.  Of course, we've been there before, but on this day, everything we saw was NEW to us.  That was a treat!

There's an outdoor German Christmas market called Christkindlemarket down in 'I-forget' which square but it is really quite something.  There is German food and fare and you can find LOADS of Christmas treasures all authentically German-made and German-inspired.  Beware, though, many items are quite pricey in response to the very intricate and detailed craftsmanship.  

After walking around for a while, we found this UH-MAZING tea shop!

Argo Tea ... what a great place.  It's so great because it is exclusively a tea shop ... not a drop of coffee ... HALLELUIA!!!

It was perfect after being outside in the cold ...

Would you believe ... this place is ONLY in the Chicago-area?  There are about ten locations but ONLY in the Chicago-area.  Ahhh ... well ... more reason to travel south of our border.

And this little dude ... 

The afternoon brought us to the reason we went to Chicago  ...  the consultation with the Plastic Surgeon.  Rees was very brave and very matter-of-fact ... 

... he knows the drill and new-and-strange-doctor or not ... he was ready!!  PROUD MOMENT for sure!!

(I mean, ... look at those photos.)

Nothing has been decided but the visit went well and we learned even more about what Rees would require surgically.  I brought my trusty binder with me and was able to provide the doctor with loads of information, as well as surgery notes and his scans. 

The doctor left the room to go review the scans and came back a while later.  He was impressed with what our former surgeon was able to do inter-cranially.  And then he told us that Rees has no "lining".  There is medical terminology for the various layers of tissue, however, I don't quite understand them and he used the word "lining" to help us understand that is what he needs first.  Giving Rees a nose will not be a one-and-done surgical process.  It will likely be three surgeries.  The first surgery would be to create "lining" for him and this would likely be done by taking skin/tissue from his wrist area and grafting it to his nasal area.  The second surgery would be to provide nasal support via cartilage grafts and other supporting tissue.  The third surgery would be via a forehead flap that would create the outer layer of the nose.  The whole thing makes us nervous but he explained it in a way that indicated his expertise and confidence in the process.  He also mentioned the space between Rees' eyes and inquired as to whether or not we were going to have more surgery for that.  We said that we were looking into it but hadn't decided anything since the retirement of our former surgeon.  He said that he wanted us to have a craniofacial surgeon designated because he would not want to move forward with the nasal reconstruction until a plan is set with and he is cleared by a craniofacial surgeon.  

We told him that presented a problem for us as we have been undecided and troubled by finding a surgeon for Rees.  We asked if he knew any ... and he said FOR SURE he did.  I know, ... our question probably sounded odd.  So, there are a couple of surgeons in the Chicago-area (he said he knows others further away but that these are a good place to start).  He is going to contact and share with them the information about Rees.  He took several photos and will prepare a tentative plan and discuss with the other surgeons.  

So, I guess that's where we're at.  We are thankful for options ... even though we're not used to it.  We're thankful that Rees is in a good place and nothing is an emergency at this point.  We're thankful for the great day we had in Chicago and that our kids were troopers and had fun mostly with just walking around.  There is so much to be thankful for and we hope you all do too!  

Happy Thanksgiving from us to you!!    

The Adoptiversary!!

We've just celebrated the 2nd Adoptiversary of Jonah and Rees!

What a difference in SO MANY WAYS! 

Two years ... it's such a short amount of time and yet, it seems like's it been longer than that, especially since SO MUCH has happened since these dumplings joined our family.

Jonah and Rees have grown SO MUCH and have gone through SO MANY changes, physically, emotionally, and intellectually!  The transformation is nothing short of amazing!

It's been a wild, crazy ride, one that I have admittedly wanted off of a time a two ... But undoubtedly an adventure orchestrated by God and I thank HIM that He's kept me in my seat!

So, we watched a movie and they chose Penguins of Madagascar.  Not what I would have picked ... LOL! ... but I was just thankful for the comfy-family-bonding time *and to get off my feet*!  The best line of the movie, (that I'm going to try hard to remember so I could use it on one of my dumplings - I just know the opportunity will arise) ... "Whoa! Down-back the moxy, sas-mouth!"

Gah!!  I love it!!

Rees has just been diagnosed with Von Willebrand's disease, a blood disorder that affects how the blood clots.  This is likely the culprit for all the issues with excessive blood loss during his surgeries.  So, we were at the hospital the other day testing out a medicine that can be given prior to surgeries to help his blood adhesion and clotting factors.  We had to get stuck twice ... *OUCH* ... (and when I say "we", of course I mean HE). But, hey, it's hard on mama too.

All was well when he was brought food, toys, and the remote for the television!  The best thing to Rees about going to the hospital is getting to watch nearly unlimited Disney Jr. and Nickelodeon Jr. Stations and playing on the iPads.  

The medicine was administered intravenously, and then at the one hour and two hour marks after the medication was in his system, blood was drawn so his factor levels could be evaluated.   Now we wait ... for about a week, until the results come back.  Hopefully, the results will show that he responded appropriately to the medicine.  If he does, this medication will be administered prior to surgeries to prevent all the bleeding issues.

Never a dull moment ... that's for sure!  But we are thankful for the answer to this problem and hopeful for the solution ... God is good and Rees is doing well with all of his healing and growing these last few months.  

Always something to be thankful for ...