October/November 2009

Dorian using Wynn's hand as a chew toy.

This little cheerleader for the boys' team is NOT happy she got stuck being escorted by Ben and Alex at homecoming!! Priceless face!

Happy Birthday to Dorian and Wynn!!

It’s been a fun fall for us at the Johnson house. Lots of milestones, celebrations and fun for all.

Dorian and Wynn celebrated their 1^st birthday on October 27^th. It was a day filled of memories, hugs, and hope for the cutest little babies on the planet. The weekend before their birthday we had a little party for them. They didn’t particularly like cake or icing, but they soaked in all the attention and everyone snuggling with them all weekend.

On that same weekend our neighbors hosted a Halloween party and they gave us the chance to raise money for the MPS Society at the party. What an amazing time that was! Everybody came in the funniest and most creative costumes, and everyone had an awesome time. We were humbled by the generosity of those that attended the party and our friends that made online donations to the MPS Society. You guys don’t know how much it means to us.

I made an awareness video in late Septmer to raise reaserch dollars for the MPS Society. Here is a clip. Please feel free to pass it along to all your co-workers, friends and family members. The more money we raise for the MPS Society, the more chance we might have in finding a cure for ML II. http://www.youtube.com/watch?v=zM5xQymjNpU

If you donate, please do it in honor of Dorian and Wynn and specify “ML II” on the online donation form at MPSsociety.org.

Stay tuned for more party/fundraising events with us in the spring. We are tinkering with the idea of hosting a poker tournament in the spring, or some other event to get together with friends and raise some money for the good folks at the MPS Society. Fun for a great cause! How can you beat that?

Both babies got their heart meds doses increased since they are a little bit bigger now. Dorian weighs 14.1 pounds, Wynn weighs 12 pounds. Dorian is quite a bit taller than Wynn which makes up for the heavier weight. They’re on their 2^nd round of synagis shots (RSV prevention) and are doing well with that. They got their regular flu and swine flu shots (as did Ben and Alex) so now we’ll just pray we all stay flu free this season. Whenever we see you next, Chris and I will be more than happy to do a flu mist/tackle Alex- to-the-ground re-enactment as he kicked and screamed for you. It was ridiculous, but kinda funny looking back.

We have put in a request to increase the amount of physical therapy the babies receive, as well as start speech therapy. Our pediatrician and our auditory therapist recommended it to help the babies not just speak but also it helps them with learning how to chew/swallow well. Wynn does a great job of scooting around on his tummy on the floor. One time he was cruising so good that he played hide-and-go seek with Chris under the leather ottoman/coffee table. Chris didn’t know Wynn wanted to play that game so when he looked around, he was like “Where’s Wynner?” and we found his little legs sticking our from under the ottoman. It was pretty darn funny. Dorian isn’t as mobile just yet. He is much more laid back than Wynn is. In fact, Wynn amuses us for his high maintenance-ness. Dorian is really chill and we are working on him to be a little bit more curious about his surroundings and reach for toys and scoot a little better. Since his joints are very stiff, we assume his lack of desire to move around is because it’s probably painful for him at times. His range of motion is more limited than Wynn’s too. So we are working on increasing his flexibility and making him feel as comfortable as possible while still challenging him to use his body as well as he can.

Ben and Alex continue to be super studs at football. Their team, the Colts, went undefeated all season and were ranked #1 until the championship game this weekend. Next weekend go to the flag football Super Bowl (called the Cougar Bowl) and play the runner-up city champs of Carrollton. Time to get face paint for the babies and cheer on their big bros! I have some pictures here of their homecoming they had last month, it was too cute.

Well thanks to all for checking in on us on occasion and for those that feel like donating a little something to the MPS Society in honor of Dorian and Wynn. We are grateful for so many things. Friends that we have fun with, family we can vent with, the fun things Ben and Alex do, the heartwarming smiles and excitement the babies have in their eyes every day, for Anna being a big help to our family, and for the parade of angels we have coming through the house on a weekly basis (PT, OT, Auditory Therapy, 2x week home respite care, etc). There is big truth in the African proverb “It takes a village to raise a child” and we are so grateful for our awesome village!!

Fall 2009

Having fun at home.

Going on their daily walk.

Handsome boys on their 1st day of school!!

Hope all are doing well. The final month of summer was super busy for us all. Ben and Alex started a new football team, Chris and I did many projects around the house to get it ready to go on the market, our contractor Mike remodeled our master bathroom, Chris got foot surgery (on his little toe, he's getting better), I went to NY to shoot a film crash documentary for TLC (should air sometime late October) and Ben and Alex bid farewell to summer and started kindergarten. So thanks to all who have been patient and withstood our unreturned phone calls and slow email responses... we are still here!

The house is much quieter now that Ben and Alex are gone at school during the day. I'm sure the babies miss the fun commotion. Sadly I kinda do too. Ben and Alex went to the babie's geneticist Dr. Cramer a few weeks ago. Since both Chris and I are carriers of the ML II errant gene, Ben and Alex have a 50% chance of being carriers of the gene like us. Their results came back that indeed both of them are just like Chris and I -- they also are carriers. That does not mean that they will get sick. It means that if they have children with someone else that is a carrier (1 in 100,000 chance) they will have a 1 in 4 chance of having a baby like Dorian and Wynn that are affected by ML II. So we'll be keeping the results and once they go down the road of having children, it'll be something to discuss with the future daughter-in-law to do some genetic testing. By the time that happens, there will be so many more medical advances that who knows if ML II will even be a problem anymore. All I know is that when it came time to draw their blood, Ben was a warrior -- very tough, barely flinched. Then all decibel records were broken as Alex was howling like he was giving birth when it was his turn. We told them they had to have their blood drawn to start kindergarten, then we took them straight to McDonalds afterwards as a reward. (yes, sometimes lying is justified!)

Next month the babies will start some Synagis injections to help protect them from RSV. Synagis is given to preemies or to children younger than 2 who have heart diseases or pulmonary diseases, or congenital airway abnormalities. Hopefully this will keep them healthy through the cold/flu season. We'll be taking extra precautions of all of us getting flu shots and very diligently hand washing, etc. We'll find out from our doctors also if we need to get swine flu shots.

The babies visited our orthopedic doctor, Dr. Brown and we were happy to see that their bones have gotten stronger (calcified) and the bowing in their legs is less severe in both babies. Dorian still has one leg that is pretty bowed but it's not as bad as it was last time and we'll just keep a close eye on it with our doctor. Wynn continues to get weekly auditory therapy here at home, and both babies have twice-a-month physical and cognitive development therapy here at home as well.

Other than that, we're waiting to hear this week if we got the foreclosed home in our neighborhood that we bid on. It's a little bit bigger, with a guest room for visiting friends/family, and has a pool in the backyard. Which is why we put the house on the market in case we need to sell this if we get the green light. It's kinda hard keeping a house with 7 people clean enough for house showings, but we're all pitching in so it's been good. Hope everyone has a great month!

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.  

Summer here we come!

Dorian looks so grown up in this picture!!

Wynn loves to hear his voice and he always has something important to say.

Dorian and Wynn enjoying their 1st movie experience!  All smiles of course. 

Wynn is thinking "Holy water is cold papi!"  

Dorian didn't skip a beat with his pacifier during his baptism at all.  

It's been an action packed month for all 4 Johnson boys.  Ben and Alex finished up preschool yesterday and sang for the Kindergarten graduation (very well choreographed I might add!), and they are finishing up football season (today is the championship game and I'm smellin' a victory!).  

Dorian and Wynn also had a busy month!  On May 16th we baptized them at St. Catherines of Sienna, the same church were Ben and Alex were baptized.  It was great to have friends and family from near and far help us celebrate our big occassion for the babies.  Thanks to the Godparents Kiley and Stacy for your support, and for everyone who was there that helped make it a special memory for all of us.  

The babies have enjoyed many "firsts" this month.  They went to go see a movie with the big boys.  They actually lasted 50 minutes in the theatre before I wheeled them out in the stroller and walked around the mall with them til the movie was over.  They did a great job!  They also went to their first pool party.  Although they slept through most of it, it was still fun to take them to the annual memorial day party we always go to.  

The babies are going through a round of antibiotics to clear up the considerable amount of fluid in their ears which we did not know they had.  The ENT caught it during Wynn's hearing aid check-up visit and since Dorian was there he quickly examined him too.  Both of them have extremely narrow ear canals so a pediatrician with normal ear scopes would not see it.  We'll see how much the antibiotics can help clear out to hopefully delay putting in tubes.  The great news out of all of it was that 25% of Wynn's hearing loss could possibly be reversed with the removal of that fluid.  Even though he would still need to wear hearing aids, there's a chance it wouldn't be as severe as we initially thought.  

During the 1st half of June the babies will get checked up by lots of doctors so it'll be a good barometer of how they are doing in all sorts of areas (lungs, eyes, heart, skeletal, etc).  

Summer is filled with all sorts of fun stuff - we are leaving for San Antonio tomorrow for our 1st family vacation with the babies to visit family and see all the kid-friendly sights (Sea World, Six Flags, the River Walk, etc).  Ben and Alex will be doing swimming lessons, Ben's playing  basketball on the team Chris is coaching, and we'll be scheduling tons of play dates since they are home with Anna and I all summer.  Wynn will be starting he speech/hearing therapy through ECI in June so I'm sure he'll dazzle them with his cuteness.  We'll step up our physical therapy to get the boys to do the fun stuff like rolling over (Dorian is almost able to do it on his own!), sitting up (we still have a little ways to go on that, they look like they are drunk when we try to do it with them), and bets are on to see if they'll say "mama" or "dada" first.  Yes yes I know, they usually say dada first since Ds are easier sounds to make for babies, but a girl can hope, right?!?!  

Today Chris and I are celebrating our 11th year wedding anniversary!!  It's amazing how quickly it's flown by, all the fun things we've done, all the places we've visited, and the pack of kids we've had.  Who'd a thunk it?  Alot of you that read this were there for us on our special day 11 years ago... thanks for sticking with us through it all!  Looking forward to seeing as many of you as we can this summer!  Dallas is always a fun place to visit so please come on down! :) 

May 2009

Dorian... quite the happy boy!

Anna with the boys.  I'm sure she thinks we're crazy!  

Only Wynn can make a hearing aid look good!

Hi everyone!  This month has been a busy one!   Thanks for everyone's patience as we have a tendency of not being great at returning phone calls.    This Saturday we welcomed Anna from the Ukraine as our Au Pair.  I told Chris that I was gonna buy a bottle of champagne and pop the cork as soon as she stepped into baggag claim -- and it wouldn't be for her, it would be for ME!!  We are so happy that she has joined our home sweet home.   I've been happy at how Ben and Alex have taken her in very naturally.   I even found out after her 3rd day here that Alex got into a heated discussion at school defending Anna because one of the kids in his class was saying Alex wasn't telling the truth about how she makes really really good paper airplanes.  How cute!  She is a tremendous help with the babies and keeping the house in order with all of Ben and Alex's stuff... it's like a dream come true.  I look forward to our friends all meeting her, she is very nice.  Here is a picture of the boys with Anna tonight before we all went to the store.  Notice the welcome signs they made for her in the background.  :)

Wynn is officially wearing his hearing aid everyday.  The right one doesn't fit well into his ear so it's being remolded and should be ready within another week, but he wears the left one when he is awake.  We have a hard time with the ringing sounds the hearing aids make, but the audiologist assures us we need to just wiggle it in deeper into his ear and the ringing should stop.  A whole new world has opened up to him now that he can really hear.  The profile picture of Wynn above shows his left ear... cute, huh?  

Dorian and Wynn had their 6 month check-up this week with another round of vaccines.  They didn't like it at all, and Anna couldn't watch them get their shots.  Both babies rank under the 5th percentile in height and weight but that is to be expected with their condition.  Both little guys weigh 11 pounds.  Next month we'll be busy with lots of doctors appointments, we'll be seeing some new specialists that were recommended.  Not because anything is going "wrong" at this point, but because we need to get the babies introduced to the folks we'll be depending on in the future (cardiologist, ophthalmologist, and pulmonologist).  

We've heard through the grapevine that the Children's Hospital in Denver is going to have an MPS Awareness Day with some events planned around it.  We're trying to find out more information about it and possibly may pack the kiddos and go out there.  We have good friends, the Simpsons, that live out there so we'd have fun visiting them and also enjoying everything Colorado has to offer.  But there's no definite plans, we just want to start getting involved with different ML II research and awareness foundations out there.   You never know when a new study is going to pop up and it would be nice to have the babies be known in the ML II research community. 

One of our friends asked us in lieu of gifts, what foundation we'd like donations made to.  Although we haven't really picked one, and we don't plan to start our own foundation, we want to affiliate ourselves with one this summer.  The one I've been reading up about a bit this month is called the Hide and Seek Foundation (that supports all lysosomal diseases).  Their website is www.hideandseek.org.   More information to come in the next few months... stay tuned. 

Wishing everyone a safe and enjoyable month!  

Hi guys!  I finally figured out how to enable the "comment" feature in this blog, so if anyone would like to post any comments, please feel free to share your thoughts with your fellow Dorian & Wynn groupies :)  Thanks for all your well wishes and positive thoughts!

Today Wynn had his 3rd hearing test.  It was a fully sedated test where after he feel asleep from the anesthesia, they connected a variety of probes across his head, and sensors within his ears.  For an hour while he slept they did a variety of different sounds/pitches on the machine and measured his brainwaves and ear drum movements in response to the sounds.  Unfortunately, Wynn's ear drums and brain waves showed that he is unresponsive to a wide variety of sound pitches and volumes.  

I told the audiologist I wasn't sure that was accurate because he coos and "talks" with us all the time, and is very responsive to our voices when we walk into rooms, etc.  But she told me that he is overcompensating with his other senses.  So when we talk to him, he is responding to our facial expressions.  If we walk into the room, he is responding to our movement.  And if we whisper to him, he isn't responding to the whispered voice, he is responding to our warm breath that he feels in his ear.   That just makes me want to hug and squeeze him even more!   He is so smart!  

She gave me a chart that plots out what his hearing ability is.  Examples of what he can't hear would be the humming of the refrigerator, high pitched voices, birds chirping, "inside voice" conversations.  What can he hear?  Really loud talking, crickets in his room (I guess if biblical infestations happened to hit our home), vacuum cleaner, motorcycles, headphone music with volume cranked all the way up, etc.  But please... if you come to visit us, please don't yell at him (!!).   Just because he can't hear you doesn't mean he isn't bonding or understanding you.   He is our little social butterfly and he'll talk your ear off, even if he can't hear his own self.  He loves to talk.   Kinda reminds me of Alex.  

So we will be getting him a hearing aid as soon as the results are shared with our pediatrician and our metabolic specialist.  The sooner we get him his hearing aid, the better chance we'll have at not interrupting his ability to learn how to speak at this very crucial time of speech development.  The results will also be shared with ECI (the early childhood intervention folks that come out and do physical and cognitive development therapy twice a month).  Now that he is officially hearing impaired, ECI will also have someone come out and do auditory therapy as well.  So I'm glad we found this early enough to do some proactive intervention.  There's no doubt that Wynn will make his hearing aid look cute as can be.  For Halloween I'll just dress him up like a mini-secret service agent in a dark suit.  Problem solved!  :-)