June flew by!

We all had a fun time at the NICU reunion at Medical City.  Here is Dr. Treen, our neonatal doctor that didn't give up on finding a diagnosis for the babies.   

Dorian flashing smiles while on my lap

The babies getting ready at the outpatient surgery center to get their tubes put in.  Aren't they cute in their hospital gowns? 

Ben and Alex in their Summer or Bust mobil.  They had fun with it for about 2 days then just destroyed it flat so I'm glad I took a picture of it.  

We had a good time and busy schedules in the month of June.  Chris was busy going back and forth up north on business and to visit family/friends, Ben and Alex did swim classes, and Dorian and Wynn went to tons of doctor visits.  

Wynn has discovered how fun it is to yank his hearing aids out, so we're working on that.  Both babies had tubes put into their ears.  Both babies, but especially Dorian, have very narrow ear canals and they both had a ton of fluid built up in their inner ear.  They were champs in the outpatient surgery center that whole morning, they were great.

We met with our new pulmonary doctor, Dr. Galfand.  Our visit with him was incredibly helpful.  He doesn't look much older than Chris but he has actually had much experience working with children with lysosomal diseases, so he knows exactly the things we should be vigilant about in the coming years and explained to us many of the tough decisions we'll be faced with moving forward.  It all boils down to how much invasive intervention we'll do once their airways begin to constrict.  We realize they'll need help in the breathing department sooner or later but we're thankful it's not needed now.  But this doctor also gave us many other valuable resources we'll need to consider in the future so it was comforting to know he has other patients who have gone through very similar progressions.  

We went to Scottish Rite again to have the babies' overall ortho doc take a look at them, last time we saw him was a few months ago. The only thing he did say that was new is that Dorian's curved leg bones are quite severe and if they don't get better within the time a "normal" child would begin to walk then we'll need to surgically straighten the bone.  No braces since that would not help at this stage.  A bone so curved would not be able to sustain weight properly, so he would have a very difficult time learning how to walk.  

Yesterday the babies saw Dr. Shiffman, our metabolic disease specialist.  Nothing really new with this visit, just a standard update-the-doc visit.  Interestingly he told us he discussed the babies with a renowned genetic expert that did much of the early groundbreaking studies on ML II in St. Louis recently, so it's good to know he keeps very current with anything that could be happening in the research field of this disorder.  

Ben and Alex spent a few days in Nebraska with Chris's sister and parents last month.  They had alot of fun visiting places, playing and hanging out.  It was too quiet for me while they were gone.  I think even the babies missed them and all their noise!

Our start of July has been great with a fun neighborhood 4th of July Festival... Ben and Alex had a blast playing with their friends at the lake and Dorian and Wynn were sweet as can be and lasted all the way til the last firework (nearly 3 hours! we were pleasantly surprised since they get so sweaty and uncomfortable outdoors normally. Yay!!).  

Wishing you a fun July!!  

Summer Vacation and June updates

Dorian and Mami waiting for big bro's to finish the big roller coasters

The four Johnson boys partying it up at the hotel on the River walk.  We can get used to this!

Ben and Alex cozied up and feeding the dolphins at Sea World.

The Shamu show at Sea World was so beautiful it made me cry!!  Check out the two whales in the background.  Just breathtaking!!  It even made Wynn drool he was so mesmerized!  

Wynn in his old lady sun hat while big brothers continued the roller coasters at Sea World. 

This first week of June has been a busy one.  Ben and Alex's football team won the city championship as we predicted.  It was an action packed game that went into overtime.  They had alot of fun. 

Then the next day we headed to San Antonio for our family vacation.  Ben and Alex had a great time enjoying the roller coaster rides at Six Flags Fiesta Texas, seeing all the beautiful animals at Sea World, and playing with their cousins.  They even got to hand feed the dolphins!  We quickly learned that the babies do not enjoy riding in the car for more than 20 or 30 minutes.  They cried for 4.5 on the way to San Antonio and cried 4.5 hours on the way back home (nonstop. literally!) .  So Chris and I are certain that no trips up north to Kansas/Missouri/Nebraska will be in our immediate future since it was torture on the poor little guys and 6 or 9 hours of nonstop crying isn't good for them and it certainly isn't pleasant for us either.  

Preschool is officially over for Ben and Alex so their first week of summer vacation was a good one.  Lots of playing with toys, swimming at the pool and going to the park.  They also went with us to Scottish Rite Hospital for the first time for Dorian and Wynn's hand clinic check up.  We are laying the groundwork about slowly exposing them to Dorian and Wynn's health issues, so we prepped them on the way to the visit by saying "All kids are not the same.  Some kids bodies are made different.  They may be in a wheelchair, or not be able to use their arms, but they are just like you-- they like to play and laugh."  So before we parked our car I warned them "You might see kids in wheelchairs, or kids that walk kinda funny, but remember they are just like you."  It hadn't been 2 minutes that we were inside and a little girl was in a wheelchair with her head in a device that held it up by ropes and a chin guard.  Alex points to her with a big smile and said "Look mami, just like you said!!"  I quickly looked at Chris and luckily the little girl was sitting in front of a big fish tank so I responded "yeah!  I told you there were tons of cool fish here!"  Situation diffused!  Babies had their hands examined and their hand splints have been doing a good job of helping extend their thumbs.  Dorian needs more stretching done to continue to loosen up his stiffness in his hands but he has improved a great deal since the beginning.  Wynn's hands are doing great and I'm not even sure if he needs the hand splints anymore.  

The ophthalmologist office visit went well.  After getting their eyes dilated and cornea's checked, there was minimal clouding so that is good news.  Dorian will start wearing a patch on his eyes a few hours a day to help with his crossed eyes.  I thought it would be like a pirate patch, but it's not.  It's just like a eye shaped band-aid.  So no arrrrgh's please.   :)

The visit with the cardiologist gave us a little reality check.  Both babies were placed on heart medications after getting their ekg's.  Dorian has "mitral valve insufficiency" which is when the valve between the upper left chamber of the heart (atrium) and the lower left chamber (ventricle) does not close well enough to prevent back flow of blood when the ventricle contracts.  Wynn in contrast has "aortic valve insufficiency" which is when the aortic valve becomes leaky, causing blood to flow backwards into the left ventricle.  They both have mild versions of these conditions so it's good that it was caught early and that we're able to intervene medically instead of anything invasive like surgery.