Wednesday, December 23, 2009

Only Ben can have this much fun refilling his baby brothers' breathing treatments.

Dori resting up at the hospital.

Picture time with the boys on my birthday.

Merry Christmas!! Feliz Navidad!! Happy Honda!!

According to Ben and Alex, Happy Honda is a fun holiday. After much probing I finally figured out they meant Kwanza. Oh.

It doesn't matter what the holiday is, we have alot to be thankful for this Christmas season. Ben and Alex had a great 1st semester at school. Alex capped it off with performing in School House Rock. He did great with all the singing, dancing and what not; but when he'd get tired he'd take a knee in the back row. At the cost of looking like Jon Bennet Ramsey's stagemom, I quit motioning him to stand-up and we just enjoyed the performance as Benny cheered him on.

Ben had fun showing me off at his first Kindergarten fieldtrip :). I was chosen in the drawing as a chaperone and of course Ben was in my group. First time most of these kids have ever ridden a school bus so all the kids were so excited! Turns out I suck as a chaperone. Before we even left for the field trip, I had already lost my own child. Ben had boarded Alex's bus without my knowledge. I have a pretty good feeling my name might not be drawn again for chaperoning duties!!

Dorian gave us a little scare 2 weeks ago. His cough and cold wouldn't go away so when we took him to our pediatrician, she told us to immediately take him to the hospital. "Don't stop off at home to pick things up, go straight there." So inside I know both Chris and I were freaking out but we have a good ability to not let the other one know. Dori was having a really hard time breathing, his heart murmur sounded worse, he had stopped eating and we were afraid he was getting dehydrated. After 5 days of waiting for his oxygen levels to stabilize without him needing to use oxygen in his nose, and after he finally started eating on his own again we were finally given the green light to go home. It was a viral infection that took it's own time to work itself out. Chris and I alternated shifts and one of us was always with Dori at the hospital because he hates to be alone. Ben and Alex really missed him. Dori really missed Wynn too because the whole time he was in the hospital, he never smiled and didn't want to play at all until the final hours of the last day.

This month has been a tough one for our extended ML II family. First we lost Carter "Boo" Brotherton. That first week the babies were diagnosed with ML II, Chris poured into the internet to find any/all info he could. He came across Carter's blog written by his dad. They were the first and only family on the planet we had the chance to reach out to and ask questions. With much empathy and kinship, they would always provide us advice, helpful info, and general encouragement. By just watching Boo's pictures, reading about his adorable adventures and watching his fun home videos, we fell in love with the cute little guy. We are so sorry for his dear parents for losing their sweet angel. Carter just turned 3. It hit us really hard. Then just two weeks later, another ML II mom (single mom) with 2 ML II kids lost her daughter, Olivia "Cookie" Armand. If you ever watched any of Cookie's videos, you could automatically see that she was full of spunk and spirit. She was a hoot! Always smiling, laughing, scooting around on her special bike, and always playing with her baby brother Mikey. Olivia was a few days shy of her 5th birthday. Her mother Trish has always made me think how much harder of a road this would be if I didn't have my Cito. Aside from those two new little angels, there were also 2 other ML II babies that passed away all in the same week. It's a sobering look into a dark window that someday we'll have to go through, but it's comforting to see the amount of prayers and support these strong parents have been offered from loved ones far and wide.

We wish all of you a wonderful Christmas and look forward to sharing more with you next year. Come on down to visit us in Texas whenever you'd like ... we love visitors! Have a blessed holiday.

Monday, November 16, 2009

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 1st birthday on October 27th. 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.

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

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 2nd 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!!

Wednesday, September 2, 2009

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!

Monday, June 29, 2009

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!!  

Saturday, June 6, 2009

Summer Vacation and June updates

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

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.  

Friday, May 29, 2009

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! :) 

Wednesday, May 6, 2009

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   More information to come in the next few months... stay tuned. 

Wishing everyone a safe and enjoyable month!  

Wednesday, April 1, 2009

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!  :-)

Tuesday, March 24, 2009

Spring: The Gift of Perspective

We are happy that spring is around the corner.  Ben and Alex no longer feel trapped inside the house all the time, football practice has started three days per week, and we're enjoying March Madness on TV.  Dorian and Wynn are enjoying the stroller rides we take around the neighborhood and listening to the birds chirp when we're outside.  

Dorian and Wynn turn 5 months old this week (on the 27th).  As they get older, we have fun listening to them coo and observing how alert they are to the world around them.  Alex likes to make Dorian laugh and Ben enjoys giving them quick hugs.   

Dorian took his second barium swallow test at the radiology department last week.  The OT and the doctor were pleasantly surprised since he did not aspirate (choke) on any of his liquids or solids they gave him.  So he no longer needs to have his formula thickened, he can have normal consistency for his formula/breast milk and we've started both of them on baby cereal.  

Our challenge has been finding an au pair.  The two last au pairs we chose we were both very excited about.  Both were nurses by training, and were very eager to join us here in Texas.  But unfortunately, both au pairs had their visas denied by their home countries (one from Brazil, the other from China).  Thankfully, Cultural Care Au Pair (the agency we are going through) has been good at sending us more applicants, and we chose a new au pair last week.  She is a college student from the Ukraine, and we are hopeful her immigration appointment next month will go well and that her visa will be granted to come to the US.  If so, she'll be with us by May 1st.  

Today the babies saw their neurometabolic specialist, Dr. Schiffmann.  When we entered the room, the babies were the center of attention because Dr. Schiffmann was accompanied by four other geneticists.  They were all really nice and Chris had an audience for his one man comedy routine.  :)   The doctor told us he is pleased to see the babies made it through winter without any major respiratory infections or setbacks.  We are not going to move forward with enzyme replacement therapy.  Although that treatment is helpful for children with other lysosomal storage diseases, those other diseases are concentrated in only one malfunctioning enzyme.  With ML II, it's 15 different enzymes that do not work properly, so the weekly IV infusions would not make much of a difference for either their neurological or physical issues.  

Both babies were very social with Dr. Schiffmann and his nurse -- laughing, following his face and all his toys he would take out of his old fashioned doctors bag.  They were a hit with everyone in the room!  We were so proud of them. 

As the months pass, we notice how tiny the babies are compared to "normal" babies their same age.  Our doctors assure us they are eating and growing at a healthy rate considering their condition.   But any babies also 4-5 months old look like sumo wrestlers compared to Dorian and Wynn.  That's ok.  I no longer get my feelings hurt when people at stores ask how old the babies are and gasp "Oh my goodness!  They are so small!"  I'd probably blurt out the same thing I guess if I didn't know any better. 

Although they are behind on some physical things a five month old "should" be doing (pushing up with their arms and rolling over), we do not stress about it.  I reconnected with an old friend from college who has a son with downs syndrome.  She told me something that really changed my outlook.  She said once she stopped comparing her son's abilities (or lack thereof) to other kids his same age, she was finally able to be at peace with his condition and actually enjoy the things he could do, instead of being sad about the things he couldn't.   So that's the mentality we've adopted.  We look forward to all the fun things spring has in store.  The babies will be baptized in May, Ben and Alex will enjoy their first real summer at home, and (CROSS YOUR FINGERS!) our au pair will finally arrive in May and make our lives much easier.  

Make sure you come on down and visit this summer everyone!  We love the company. 

Thursday, February 19, 2009

We've had a good past couple of weeks with all the boys.  Ben and Alex celebrated their 5th birthday at the bowling alley.  They led their friends to the Cha Cha Slide with the party hostesses and had a great time.  Dorian slept through the whole entire party while Wynn was wide awake the entire time.  

About two or three times a month we have therapists from ECI visit the babies here at home.  Our doctors recommended enrolling into the ECI program - a home therapy program for families with developmental and physical special needs.  Kris is our physical therapist for the babies, and Joy handles their cognitive development activities.  Joy came over and Ben and Alex were still home and Ben kept on circling around her while she was holding Wynn very protectively like "who are you and what are you doing to my brother?".  At the same time, Alex was telling her which baby was which just in case she felt confused.  It was funny. 

We did an introductory appointment with Dr. Brown, a pediatric orthopedic specialist who has had experience working with kids with storage disorders.  I was touched by his story on how he got into this specialty.  He was born with only fingers on his left hand, so he'd volunteer at Scottish Rite with for the physically challenged kids.  As a volunteer, one of the hand doctors took him under her wing and encouraged him to pursue medicine.  

Both babies have bounced back from a respiratory infection they had last week.  Between the antibiotics and the nebulizer (for breathing treatments), it took them about a week to get better.  

Wynn is a big ham and likes to smile alot now ... I guess he didn't want Dorian getting all the cheers from us every time he smiles. 

Sunday, February 1, 2009


We've been very grateful on how generous our friends have been to us.  We are so blessed to have so many great people in our lives.  I realized today as I looked around during this 1st half of the Super Bowl game.  Dorian and Wynn are wearing new outfits given as gifts, Ben and Alex are watching a Tom and Jerry dvd that were given last week, I drank a sip of Kahlua shipped from friends in Maryville (yes... they know my kryptonite), and Chris is enjoying the company of one of our neighbors who invited him over to watch the super bowl.  

From our friends that have been to visit (both our friends in town and also those that have come from far away), the Phi Mu's that continue to spoil us with yummy dinners, the play dates friends have arranged for Ben and Alex, and all the thoughtful calls, emails and cards that we receive on a weekly basis -- you all have made us feel that we are the luckiest people on earth.  You all are priceless.  

Everyone has had a good couple of weeks since the last update.  Dorian has done well enough with eating his thickened formula that we pulled the feeding tube out of his nose earlier this week.  Dorian has become quite the crowd pleaser -- he loves to smile and make puppy dog eyes to Chris and I.  About a week and a half ago he started intentionally smiling so that is a fun milestone to be a part of.  He also does a great job with following objects.  He looks like he is itching to start grabbing things.  

At our last Scottish Rite appointment -- Wynn's hands and wrist flexibility had improved quite well according to the doctor.  Dorian's hands and fingers are stiffer than Wynn's, but she did see a little improvement, so we're happy.   Wynn loves to coo and he is very sociable. He likes to be talked to and cuddled as often as possible.  

The babies have been busy busy busy with their firsts.  They went to the park with their big brothers yesterday for the first time, they went to the mall when Hayley, Jaymie and Stacy came to visit, and I started prepping them for their first TV appearance they'll be doing for an interview the boys, Chris & I will do for work stuff (my work stuff) with Univision (the Spanish language channel).   I guess there is not much prepping a 3 month old needs to be on TV... just be as cute as they normally are.  So that won't be hard for them. 

So as I watch the super expensive commercials during this super bowl game, it makes me thankful that our friends make us feel like one in a million.  

Monday, January 19, 2009

All four boys had a great weekend this weekend.  My sister came into town to visit and my nephew Nick came up from Ft. Hood to spend the weekend here.  It was so nice to see Nick, we hadn't seen him since he left for Iraq 15 months ago.  The babies went to their first party on Saturday!  Between the bounce house, the snacks, and all the friends under the age of 5, our four boys had a rockin' good time.  Then on Sunday, Ben and Alex went to another party for their school friend's birthday.  So they were hungover from candy, cake and soda.  Ah... to be a kid again!

Today Dorian had a big day. We took him for his follow-up Barium Swallow Test at the hospital's radiology department to see if he was still aspirating or not.  If his aspirating was as bad as it was when he was in the NICU, he would have had to get a feeding tube surgically implanted into his tummy.  But when he did the swallow test, only a small amount of liquid was going down his wind pipe.  So for now, no need for surgery.  Yay!!  We will begin to thicken his formula with a thickening gel since he swallowed much better with it that way at today's test.  We're now able to offer him a bottle at every feeding and then tube feed the rest.  In 2-3 months we'll get him tested again to see if we can get completely rid of his temporary tube.  Wish us luck!  We were so proud of him. 

Last week we met with our pediatric geneticist, Dr. Cramer.  She noticed that Dorian's liver is already beginning to become enlarged (typical with children with storage disorders), but it's nothing to be treated or concerned about at this point.  We spent some good quality time with her in looking at what the picture is going to look like for Dorian and Wynn.   She trained with some of the pioneering genetics experts in her field, so she offers us a great perspective.  We can see that she is truly dedicated to treating children and is passionate about her specialty.  

Being that alot of these ML II issues will become prevalent near the 6 month mark, we will be busy with many doctor visits around that timeframe.  We have been referred to an orthopedic doctor that has had experience treating kids with storage disorders, so it'll be good to have a proactive doctor to help us treat the babies' bone & joint issues.  In the meantime, we have established treatment at Scottish Rite Hospital for joint and mobility limitations the boys have with their hands.  Throughout most of the day they wear splints on their hands, which makes them look like they are wearing miniature boxing gloves.  So their new nicknames during splint time is  Wynn "Boom Boom" Johnson, and Dorian "el Macho" Johnson.   This week we will begin our physical therapy house calls done by an agency our neonatologist connected us with when the babies were discharged from the hospital.  That'll be nice to have them come to us -- very convenient.  

We also will be visiting a cardiologist within the next 1-2 months to have the boys looked at and ensure that their slight murmurs have stayed a non-issue.  Dr. Schiffmann, our neurometabolic specialist, is our quarterback for all of this.  He is one of very few doctors in the country that actually has experience treating this disease and we are very fortunate that he moved to Dallas recently and set up a practice here.   He did the majority of his specialty training at Duke and furthered his research & practice with the National Institute of Health (the NIH).   When we visited him last month, we noticed that he is focused on this family of disorders since he has had experience with seeing these cases from as far back as his days of residency.   We have faith that he'll be able to keep us on track on what we need to be looking at, and what actions and treatments are best for the babies.  

We are very happy at the level of care the babies have received from the time they were in the NICU at Medical City to our team of specialists we now have all on speed dial.  Everyone has been wonderfully compassionate and professionally passionate about helping us give our babies the best life possible.

When we picked up the big boys from preschool today, Ben and Alex brought in their coloring projects they did of Dr. Martin Luther King, Jr.  I asked them "who is this guy?" and Alex said "He was president."
"What did he do?"  I asked.  
"He said it was good for Red, White and Blue to be together."
So then I turned to Ben and his response to what Dr. King did was "He was nice and he wanted everyone to have fun."
So even though the boys didn't know all the answers about Dr. King, they both knew what his legacy was about in their own minds.  It's kinda like us -- we may not know exactly everything about this complex disease -- but we know the overall picture.  One thing is for sure- the best part of this picture is having two beautiful babies that are beginning to coo and that have their big brothers wrapped around their little fingers.   

Thanks to all our friends that have welcomed Ben and Alex on play dates while Chris and I are busy at the hospital & doctor visits with the babies, to those that have brought us dinners and gifts for the babies, and to those who continue to pray for us.   We are truly blessed with our strong support system.  

Friday, January 9, 2009

Hello to all our friends and family reading our first entry!  It took me a while to think of the blog title name, but the journey we have ahead for Dorian and Wynn will be just that-- an adventure.  If you were to look at them and hold them, you'd never know that they have an extremely rare terminal disease.  

Around Thanksgiving, after a month of being in the NICU, our neonatologist warned us that she thought all their symptoms looked consistent with a very rare genetic disease called Mucolipidosis II.  When she gave us the information about the disease, she was clear to say that IF the babies had ML II, that it would "change your life forever" since most kids with ML II do not live much past their 5th birthday.  So for the next 3-4 weeks, Chris researched everything he possibly could on diseases that had the same symptoms the babies had -- but we kept on praying and hoping that it would be something else.  In fact, we were thinking so optimistically, we had basically dismissed the ML II warning from our doctor because we were confident that the babies would be ok -- maybe just have some mobility constraints due to their bone deformities found in early x-rays.  One month later, our neonatologist finally confirmed what we didn't want to hear -- that indeed the babies had ML II.  The diagnosis had finally been confirmed by a blood serum test, and followed up by a genetic test, both tests sent to genetic specialists on opposite sides of the country.  So yes, we are sure they have ML II, and yes, we have gotten a second opinion.  

Of course, hearing that your baby that you love and have prayed and hoped for has a terminal illness has been an incredible blow to us.  They are so sweet, so lovable and so cuddly, that it seems unreal that their lives will be cut short.  It's been a whirlwind of emotions for both of us -- it's unfair, it's sad, it's angering -- but it's a reality that we are getting our heads around.  

To explain it in laymen's terms, ML II (also known as I-Cell Disease) inhibits the body's cells from recycling substances throughout the body, so the cells begin to accumulate the excess carbohydrates and enzymes and fail to function properly.  Most babies get diagnosed after their 6th month of life because by then the symptoms have begun to become evident.  To our advantage, our doctors at Trinity Medical Center and Medical City here in the Dallas area knew from the day they were born that Dorian and Wynn were not normal.  The likelihood that a child is born with ML II is 2 in 1 million births.  Hence, most general practice and pediatric doctors can practice medicine for 30+ years and never hear of this condition, let alone treat a child with this illness.  

Some of the affects of this disease is limited mobility, bone and joint dysplasia, hearing problems, ear infections, pulmonary problems, cardiac problems and pyscho-motor delays.   Although there is no crystal ball to tell us how severe Dorian and Wynn's symptoms will be and which ones they will have -- the prognosis is basically still the same.  With no cure in sight and with little to no clinical trials and studies currently underway, we are not hanging our hopes on a miracle cure.  We are resolved to continue loving these babies as much as possible -- not dwelling on the the difficult road they will have ahead, but celebrating the blessing we have in them.  We have promised each other to give them as happy and full a life as possible, no matter how short it might be. 

Ben and Alex have proven to be loving and attentive big brothers.  They have no idea that anything is "wrong" with their twin baby brothers.  Since B&A are not even 5 yrs old yet, we are not ready to tell them about this disease and what it will mean in the future to them and to their little brothers.  We figure those will be discussions we'll have in the future once Dorian and Wynn's health begin to fail.  But for now, we are a regular family just hoping for the best.  

We appreciate all the love, support and prayers that we have received from our friends and family over the past couple of months.   I started this blog for 3 reasons:

1)  Chris and I felt it was easier to update friends and family via this blog than it is to make countless phone calls to update those that have expressed their concerns for staying informed about the babies.  So feel free to check in often to this blog to get the most up-to-date information on the treatments, tales and triumphs we'll face over the next few years.  Also, it's nice to keep loved ones up to date through the blog- versus each conversation eventually going to this every time we talk with people.   Even though this heartbreaking disease has consumed our energy, we don't want it to consume our lives.  So every time you call, we may just not be in the mood to talk about it. Hence... the blog.  

2)  Years down the road, once Ben and Alex are old enough to want to know details about Dorian and Wynn and what they went through, it will be like a virtual diary they can read as often as they want.  

3)  We feel it's our responsibility to spread the awareness about this disease.  We know how painful it is to learn that your child has this, and anything we can do to give attention to this family of disorders can help other families as well.  To learn more about ML II and other lysosomal storage diseases, check out  

Although I'm a writer by trade, this blog comes straight from the heart -- sometimes it'll read like I'm rambling, other times it'll sound like I'm mad or sad.  Whatever it is -- it's our life we are living and this blog will give  you a glimpse of what we are going through.  I can't promise that I will update it weekly or even monthly, but we'll give you our highlights as often as we realistically can.   Thanks to all for being our rock solid foundation throughout all of this.  

ps- i have no freaking clue how to format pictures and videos on here, so please bare with me while I learn how to use this application.  thx!