The Incredible Adventures of Dorian and Wynn

2012-01-10T17:19:00.001-08:00

Wynnie in front of his locker at school

Dori celebrating the rockin' New year

Happy 2012 to all of your friends and family who share our journey in this crazy life of ours. We hope the holidays brought you peace, health and happiness. We are hopeful that this new year will bring us many chances to see good friends and enjoy good times with our family as all the boys get older and a little wiser.

Our big highlight on Christmas morning was Ben and Alex waking us up with ALL the excitement and commotion humanly possible at 4:45 AM because Santa had dropped off their presents already. No sense fighting a losing battle, we all went ahead and woke up and were opening presents and laughing by 5:00 AM. By 7 AM it felt like it was noon! :) All four boys had a fun Christmas and have been having a nice time playing with all their new goodies.

Chris and I have been able to spend a bit of quality fun times together over this past month. For my birthday he surprised me with a evening out and a fun sleep over at a hotel here in town. It was fun and I was glad we got away for the night... it's nice to take our "mom and dad" hats off for one night and just have fun like the olden days. Then for New Years Eve we had a blast celebrating with our friends in the neighborhood the coming of the new year.

Ben and Alex had a short Christmas vacation but we were all very excited upon the return to school on Jan. 2nd because now the babies are officially schoolmates of theirs. We're grateful that the school district offers special education preschool for children age 3 and up. Dori and Wynnie qualified for enrollment into the Functional Life Skills class at Castle Hills Elementary. All the kids in school stare as we bring the lil babies into the school bundled up in their blankies, but we know the babies are loving it. They come back from their Tuesday-Thursday morning classes absolutely exhausted, but always grinning from ear to ear. All that reading, writing and arithmetic is tiring on the big fellas but we know they are learning and making new friends. Honestly, they have all those ladies wrapped around their finger. They get additional PT, OT and Auditory Therapy through the school as well.

Chris and I are really proud of the impact the babies are making to those around them. Here is an excerpt from an email their Auditory Therapist sent me... it really melted my heart:

I just want to tell you what joy your boys have brought to Castle Hills. I am so proud of you for making the leap of faith to try the school setting. I know that must have been a very difficult decision just have them leave the safety of your home and trust a whole group of new people.

I will be going in every Wednesday morning to work with Wynn. I just wanted to tell you that I think the world of you and your family! I was thinking how many people lives Wynn and Dorrie have touched and it is so amazing and powerful!

Powerful indeed! My chest is sticking out just a bit farther out, and Ben & Alex are excited to walk their baby brothers into school in the mornings. Someone asked me last week "Why do you still call them babies? They are 3 years old now." Well... that's true. Technically they aren't babies anymore, but they are always going to be our babies because they do what babies to best. They give the purest of love, they are happy to cuddle all day long, and their worlds aren't tainted with the stuff that stresses the rest of us. I'm glad to call them our babies because sometimes the best therapy for us is just to enjoy them... no pill can do that.

Well... I gotta help the babies do their long division now! Hoping you get the wild idea to come visit us here at la casa de Johnson, we would love to see you in 2012! With open hearts we hope the Lord continues to bless us and that he will continue to bless all of you as well.

2011-11-29T19:44:00.000-08:00

Cavemom and Cavedad - our annual MPS Society Halloween Spooktacular was a blast!

Cuddle bugs on the couch

The boys on halloween

Dori wearing his birthday hat

Chris spending time with Dori while hospitalized

Today while I took Luna for a night walk to burn off some of her puppy energy, I was listening to an audio book of Joel Osteen. The chapter really got me thinking because it was about how we need to focus less on our misfortunes and more on our blessings. Sounds simple, sounds obvious to "focus on the positive and the negative won't seem so bad"... but when I focus on putting my mental energy on our blessings, it truly does put our challenges into a more balanced and manageable perspective.

Three years ago when we received the terminal diagnosis for the babies, it was like getting hit by a huge truck right between the eyes. Shock, anger, sadness -- you name it, we felt it. But once we got to know our lil guys, their fun personalities and the lessons they have taught us make me realize that their "illness" has given me a healthy sense of urgency to love on them and enjoy them as much as possible. In doing so, I have been able to truly soak in their love and see what God had intended me to see through their eyes. I don't focus on the painful inevitability, I enjoy the NOW with them.

When Ben and Alex are all frenzied their limitless energy (making messes in the backyard, staying up way past their bedtime building forts in their room, or sloppily rushing through homework to get to play) I sometimes have to take a deep breath and realize that it's ok. All that energy that runs through the house like a tornado is really just their passion for life and their loyal friendship for one another. All they want to do is play, play, and play! And how can I get mad at that? They will hopefully be each others' best friends for life, so I have to remind myself that they won't be kids forever and all their craziness is just good memories in the making... and I'm just blessed enough to have a front row seat to it all.

Work for me has had its peaks and valleys these past few months. Some months are super busy with business travel and conference calls, other months are very quiet and I start to get a little nervous thinking "hey! when's my next gig! Mama has bills to pay!" ... But in those quiet weeks of no gigs, I have been able to focus more on being a more responsive mom, I've been focusing more on fitness/healthy eating, and hopefully Chris would say I have taken more time to be a better partner. Just like in any relationship (whether as a parent, a spouse or a friend) -- relationships need to be cultivated, nurtured and never taken for granted. The busy phase of life that we're in has led us to fall into the traps of neglecting the life force of our relationships. Our bonds of family, spouses and parents is a sacred bond that needs to be fiercely protected and unfortunately it's taken some wake up calls for us to get refocused on what really matters.

This past month has been filled lots of ups and downs. Dorian had a 4-day hospital stay due to a respiratory virus. He was not wanting to eat, barely talking or smiling at the hospital and just not being his normal self. On his final day at the hospital, Chris disconnected Dori from all his IV's and monitors and called in the doctor to see him. He told the doctor "Dori is going home today" very confidently, not as a question but as a statement. The doctor examined Dori and said "Yep, he has no reason to still be here." So thankfully as soon as Dori came home, it was like a switch was turned on and he started singing again, talking nonstop, and he keeps calling out for a baby since the day he got discharged. I ask him "Who's the baby? Are you the baby?" and he nods NO every time. But then little over a week after his discharge, another little i-cell baby who was hospitalized with his exact same symptoms passed away. That news hit me really hard. It was hard balancing the three boys at home, and alternating 24 hour shifts at the hospital with Chris -- but the fact that he is home and healthy now puts that stressful week into perspective. The house just wasn't the same without him those 4 days.

Alex got viciously attacked by a tree (ha!) ... he had to get 4 staples in his head after a competitive game of football with Chris and Ben on Thanksgiving. He had his staples removed today. Ben and I took him and Alex went way drama-king on us and had to be held down by 3 adults in order to have his stitches removed. He screamed, kicked and hollered and you'd think we were trying to amputate his arm. I felt so bad for him and I started to nervously laugh which turned into uncontrolled hysterical laughing--- which only made the situation worse for Alex. Ben's eyes were wide open watching this whole scene unfold so he started to cry. By then the big nurse had finally removed the last stitch and she very dryly said to Alex "Getting stitches and staples is part of being a boy. You are too big for us to have had to hold you down. I don't think you should get any ice-cream treat tonight." Way to kick a dog when he's down lady! Ben just sat there teary eyed with his mouth wide open like "oh no she didn't!". So he helped Alex up and held his hand on the way out of the exam room. That sweet Ben is such a loyal protector...loyalty is definitely one of his strongest gifts... I just love him for that!

Anyways Joel Osteen has it right... the less time I focus on what's wrong, the more open my eyes are to enjoy all that is right. It's a mental trick that when a negative thought or feeling comes into my head, I gotta replace it with a better one. So far it's working through these busy months where we've been blessed with being in the good company of friends, family, birthday parties, our MPS Society fundraiser and family outings. Thank you God for all the blessings you have given us... and for giving us the clarity for the ones you keep giving us on a daily basis!

Dragon Mom

2011-10-18T16:12:00.000-07:00

Dori having fun with Alex

Wynnie at pediatrician's office

For State Fair Day (no school) we spent the day watching movies and going to play laser tag and video games. The babies held in there for 2 whole hours!

For two weeks Viktoria is gone visiting her family in Germany and to meet her brand new baby nephew. Before she left for the airport I told her Ben and Alex would be wearing dirty underwear until she came back to resume her duties. Well it hasn't been as overwhelming as I thought it would be, but we sure do miss her tons. Dori was in his bed the other day calling out for her and laughing saying "Vika! Vika!" ... it was so cute.

A fellow i-cell mom shared this article this week from the New York Times (the link is below) about a woman who calls herself a Dragon Mom. She's the mom of a Tay-Sachs baby, another degenerative terminal genetic disorder. This is just a small glimpse of what we feel, what we aspire, and what we enjoy.

Sure with Ben and Alex we worry about their grades, their behavior and their character development as any normal parent would. We drill them to use their manners, we nag them to pick up their shoes, and we take them to Sunday School & Sacrament preparation at church every week with the hopes that it will turn them into loving, smart, generous, loyal and successful men.

But with Dorian and Wynn we focus on the here and now since that is all we have as a guarantee. Reading someone's else's perspective is refreshing and inspiring to know there is a small "fraternity" of parents of the terminally ill that all share one thing -- a rock solid and fiercely protective love for their child. I am proud to be a part of such a special fraternity ... no one ever "pledges" or wants to be in this selective group... you just end up here.

Please read this article, it warmed my heart: http://www.nytimes.com/2011/10/16/opinion/sunday/notes-from-a-dragon-mom.html?_r=1&src=tp&smid=fb-share

This month will be Dorian and Wynn's 3rd birthday so October is a month we reflect on how much they have blessed us and continue to bless us everyday.

Although we may not see Dorian and Wynn walk across a stage at Harvard or Yale to receive a diploma, we are just as proud to know that they will be starting their scholastic endeavors in January taking the Functional Life Skills program at THE SAME SCHOOL AS BEN AND ALEX!!!

The Lewisville school district designates each different elementary school to specialize in one main Special Ed program. Luckily for us, our school (just 8 houses away) offers the class that is geared toward the most developmentally delayed preschoolers. Since the babies will be 3, they officially qualify for LISD special ed pre-school. Ben and Alex are excited that the babies will go to their school with them. It makes me cry a happy-cry to know that for once all my boys will be roaming the halls and making a name for themselves on the same campus. I can't wait til January so the babies can socialize with kids and develop some new skills and routines 3x per week. I have no doubt that they will make lots of friends and melt lots of hearts in the process.

Three Cheers for Wynnie!!!

2011-09-26T17:44:00.000-07:00

Amid all the business of work, school, sports and life, Wynnie was able to slip in a nomination for his courage and bravery in his fight against ML2. Please read the latest quarterly MPS Society Magazine and flip on over to page 30 to read all about Wynnie.

He is a very cool kiddo and I feel honored that God chose me to be his mami. We love you Wynnie!!

http://gallery.mailchimp.com/191692f93c05585a3a8b323f7/files/Fall_2011_Courage.pdf

Trust

2011-09-01T22:17:00.000-07:00

Ben and Alex in St Louis

Luna Johnson

Dori in his stander

Wynnie in his standard

This summer we as a family have learned a lot about trust. Ben and Alex went with me to St. Louis for the National MPS Society Family Conference. What an eye opening and amazing time we had. Chris stayed home with the babies and had lots of fun here bonding with them. I think when I am out of town Chris is able to let his "hair" down and do things at his own groove, so that's nice that he is able to do it.

At the MPS conference, I had a sit down talk with the boys about Dorian and Wynn and their mortality. The conference had lots of sibling programs on the agenda so I didn't want some of the discussions to make them feel they were sucker punched. So while we ate dinner during our first night there, I told them we although we don't know how long Dorian and Wynn will live that we will do our best to be the best mom, dad and brothers Dorian and Wynn could ever had. We would do everything in our power to give them a happy life, no matter what.

Ben was shocked at this conversation because he just stared at me a lot throughout the course of the event. Alex started to cry and said "But I wanted them to grow to be old men with me!" I told them that miracles do happen, and we should never give up on the miracles God can create in the babies' favor. It was a hard subject to discuss, but for the rest of the conference the boys kept telling me how much they missed the babies and I could see the love they had in their eyes for the other kiddos that looked just like the babies there.

Chris and I have always told them that they can TRUST us for anything and everything. We are telling them that as long as we trust one another, we can get through anything that comes our way. Ever since we got back from the conference, they have been SO much more playful and affectionate with the babies. It warms my heart.

Chris gave in and bought the boys a dog.... we named her Luna (which means "moon" in Spanish). She is a Hungarian Vizsla and she is so cute and spunky. I pretend like it was Chris's idea and that I'm grumpy about it but I secretly love Luna so much! She's so adorable it's hard not to. The babies brace themselves when she comes around because she really likes to lick, jump and nibble a lot so they are learning how to turn their back towards her when she is coming. :)

I enjoyed my trip to St Louis and Silver Dollar City... it reminded me a lot of my childhood because I remember going to the same places with my parents on vacation. I hope the boys will always remember too. Although it would have been nice to have the babies at the conference with us, they would not have liked the 10 hour drive up there so it was best to keep them here.

I trust that those reading this will not have any mortality talks with Ben and Alex. Chris and I are really protective about what they hear and what messages they take away from a conversation -- so we are looking at ways to always be open and approachable for them, while at the same time being their main avenue for questions and concerns they may have.

The summer taught us a lot of lessons about trust:

1) trust is an assumption in its purist form... you only realize how important it is once it's actually lost. We hope that Ben and Alex will grow in trusting us, their feelings and trust in their faith.

2) Prayers are powerful. After our "mortality discussion dinner" in St Louis, Alex prayed that night that God would say "Dorian and Wynn will live to be 100, and that is how it shall be." He said it with such conviction that I have no doubt God heard that one directly himself.

3) Vigilance is so important. Whether it's being vigilant over a puppy around your babies, being vigilant over what Ben and Alex are watching on TV, or being vigilant about the world around you--- when you really love somebody or something, you will protect it with all your heart. If you don't fight to protect something, it means you really didn't care for it to begin with. So focus on what you truly love and put your time and faith into that with all your heart. Anything else is just distractions.

4) Never underestimate the power of fun. Swimming, sleepovers, movie matinees and dance parties all have curative value. REALLY they do!!

5) Waiting brings patience and anticipation. We waited all summer long for the babies to get their standers and they finally arrived yesterday! The babies can use these to put weight on their legs, become familiarized with the sensation of standing and see the world from a whole new angle. Dori loves it!! Wynnie has been sleeping both times he's been in it so we can't tell if he likes it or not. This is the first step to take in encouraging them to walk. Go babies go!!!

6) I really miss my mom and dad. There were so many times this summer where I would have done anything to call them, visit them or just vent with them. I know they hear my prayers but i truly appreciate all they did for me and the strong woman they made me that I am today.

7) I really miss Ben and Alex!! I went to the grocery store the day before school started and I saw some of Ben's favorite polish sausage. I grabbed it to place in my cart thinking it would be a good lunch idea for next week, then my eyes got all watery thinking "they'll be in second grade next week at lunch in the cafeteria, not home with me!" As crazy as they are all day here at home, those stinkers are awesome.

Public Enemy #1 and Toughness

2011-07-19T15:00:00.000-07:00

Public Enemy Number 1 - deadly crafting snakes

Ben at football camp

Alex at football camp

The babies attentively learning during speech therapy

Ben getting his "worship on" at VBS last week

Behold these slithering creatures pictured above. Our latest nursing staff dismissal was due to these wooden snakes that I purchased at Hobby Lobby as art projects for the boys. Long story short, every time she'd come and we accidentally hadn't pick up the vicious snakes, she would threaten to leave. After the 3rd time of threatening to leave and shutting herself into our walk-in pantry and calling the agency telling them she would no longer stay here if the snakes were not put away, we did our final snake sweep.

I apologized to her and ushered her out of the pantry telling her that any snakes left out of the toy baskets are not intentionally left to frighten her. She did not accept my apology and she said that the boys intentionally leave the snakes out scare her. I explained that sometimes in a household with 4 kids, not all the toys always get put away neatly. She wouldn't budge. So we just asked her to leave and we haven't asked for her back. It's sad because she has been with us since day one, but nobody can walk into our home and accuse our boys of being malicious. I'll admit, Ben and Alex are all kinds of goofy & crazy, but they aren't mean spirited... so she just had to go.

On a lighter note, the boys are in day 2 of football camp. The first day, after 3 hours in the heat and being motivated by coaches that didn't want to coddle them, they came to my car crying and saying how "mean" the coaches were. After I explained that football camp at the high school isn't for playing patty cake or ring around the rosy, they went to camp on Day 2 with a better attitude. No tears this time and they felt proud of the drills they did and the compliments they got from the coaches from working hard. A little bit of tough talking didn't hurt anybody, and I'm glad they aren't letting the boys go soft out there on the football field. We can leave the snuggling and patty-cake for the privacy of our own home :).

Ben and Alex have taken a particular interest over the past week in playing with the babies (more than usual), and it just makes us so happy to watch their interaction. They play catapult with Dori (basically throwing things in his direction and watching him roll over and get it... not as violent as it sounds!). They also like to make Dori their parrot -- telling him things and listening him repeat it. They get a big kick out of that! And they love to make Wynnie laugh by running around him. The babies have a great time, Ben and Alex have a great time, and it's just really neat to see them bond.

I'm reading Tim Tebow's book "Through My Eyes" and its soooo good!!! If you don't know his story and his background, you gotta check it out. I got a neat tip from what his mom used to do with them. She would give them "character points" anytime he and his siblings would get complimented on their behavior by an adult or if they went above and beyond and exemplified solid character. I was so excited to get it started at our home! We announced to the boys that for every 10 Character Points they earn in their allowance jar, they get $10. So for the first evening, there was ALOT of fake character going on.... "Mami you are sooo nice! Mami you are so pretty! Papi, would you like for me to get you a coke from the fridge? You are the best papi I could ever have!!!" All those compliments and offers of fetching stuff got them zero character points -- it wasn't until they forgot about it the next day when Chris took the boys shopping that they began earning them by being polite, and a waitress the next day told me how polite the boys were. Cha ching!! So thank you Mrs Tebow... The idea has been a hit at our house and hopefully they are learning some good values in the process.

Summer Snooping and the Mavs

2011-07-10T20:51:00.000-07:00

Mavs locker room

Dori at the golf course

Wynnie at the golf course

As a surprise I took Ben, Alex and Viktoria to the Mavs arena at 7:45 AM on Saturday morning to tour the Mavs facilities in downtown. Our name was drawn from a Mavs watch party we all attended back in May so we had a tour of the practice facilities, the locker room, and the weight room. I think the boys' favorite part was that the players have a lounge area all decked out in black leather recliner "theater" style seats with a huge flat screen and all the video games a kid would ever want. All these months we've been telling them video games turn your brains into mush and the world champion Mavs have proven me wrong. Thanks. They had a real fun time yesterday morning and it was neat to see.

We are big snoops in our house. Alex likes to go through cabinets, drawers, closets and any nook or cranny where he thinks he might find something interesting. I don't know what he has in mind, but he is always snooping around going through things. The other day while I was cooking he was digging through drawers and he stopped and said "Mami, I like being nosey!" and then kept on digging. I said "You know Alex, when I was your age, I used to go through all my parents drawers too." I probably should not have admitted that in retrospect! He just smiled.

In the spirit of snooping, it was 2 hours past Ben and Alex's bedtime, and I could hear them laughing and screaming and occasionally running upstairs. So I sneaked into their bathroom next to their room and just sat there and listened. Alex had his journal out and was interviewing Ben. It went something like this:

"Ben, what's your catch phrase?"

Ben responds "Skate 3!" (the name of his favorite video game)

Alex says "No, it can't be that! A catch phrase is like your favorite thing to say. Like Bugs Bunny's catch phrase is 'What's up Doc'.... get it?"

"Ooh... my favorite catch phrase is 'DUUUDE!' then... what's your's?"

Alex responds "I guess mine is 'What's up Doc' too."

I know that as this is read, it's probably not funny but while I was crouched over in the bathroom listening to this and hearing Alex scribble Ben's response in his journal, it was adorably hilarious to me.

And not to be left behind the snooping department, the babies are right with them. Wynnie has been waking up and not letting the nurses have much of a break in the evenings... all he wants to do is drink milk and chat for as long as he can get away with it, usually for 2-3 hours at times. He just loves the attention. Dorian has also been nocturnal lately. One night he just played and sang and ate starting at 1:00 AM and didn't skip a beat to take a nap until 10:00 AM the following morning after physical therapy. He's also been REALLY active in the rolling around after people. His favorite prank is to roll to the foyer and knock down all the forts and defense soldiers that Ben and Alex have built. If Ben moves him back into the family room, Dori screams at him and scoots and rolls his way right back over and knocks their stuff down right over again. I think Dori loves all the commotion it causes (1) Ben and Alex scream, (2) he hears all the blocks and stuff crash to the wooden floor and (3) he thinks "dude, I did that!". Isn't every little brother supposed to bug his older brother? He takes the job very seriously.

This summer Ben has really endeared himself to Wynnie. Our friends came to visit us last week and Ben wanted them to meet Wynnie, he kept saying "Come over and meet him, he is really really cute! He's really funny!" I think Ben is sweet on Wynnie because Ben can do all his Jerry Lewis antics to him and Wynnie just laughs and laughs at anything Ben does. Who doesn't love an audience?

Chris was out of town all weekend, so I took the older boys to the Japanese steakhouse last night for dinner. When our cook came out, Ben sat up on his knees on his chair and says like a rapper "Let's get this party started!!!" That's pretty much been the theme for us this summer... have fun and get the party started with friends, play dates, camps and everyday celebrations. So far we've had a great summer and I can't believe it's nearly 1/2 over already. ;(

Happy Independence Day!

2011-07-05T07:44:00.001-07:00

Wynnie with his favorite toy

Alex in front of fireworks

Ben striking a Lady Liberty pose

Ben getting a ball at the Rangers game!

Alex and I

Dori giving his Papi lots of kisses Friday at the restaurant

We hope everyone had a restful and fun 4th of July weekend! We had alot of fun with the boys and the babies this weekend, shared in the company of great friends. Dori spent the weekend very affectionate-- kissing and being kissed. Wynnie was in a playful mood all weekend so he had fun playing with his older brothers and the kids that came over to play.

Many thanks to the Devoe family who gave us their tickets to the July 4th Rangers baseball game! Ben got a ball from one of the Orioles players, and then Alex got a ball that Chris caught for him. They were so excited! Alex knew that the Orioles team colors would have black in it because as he stated, "Orioles sounds like Oreos and Oreos are black."

Then after the game we watched an awesome fireworks show from the parking lot. As we drove off the boys rolled down their windows and were telling people "Happy 4th of July!" "Happy Birthday America!" It was cute. Viktoria stayed home with the babies. She and Catherine, the night nurse, took the babies outside to watch our neighborhood fireworks show. Dori didn't like it one bit, and Wynnie didn't care for the heat and humidity so the girls brought them back inside and just watched the fireworks from inside the house. I don't blame them! It was still 95 degrees at 8:00 PM!

As we spent time swimming and playing in the backyard on Saturday, Alex says with a big smile on his face "This is the life!!" How true! Our time spent with friends and neighbors was just what we all needed...a fun and lazy weekend for all. We are very thankful for the peace and happiness we shared with each other this weekend.

June 26th

2011-06-26T20:34:00.000-07:00

Wynnie enjoying bath time

Dori hanging out with us in the kitchen

Ben riding his bike for the 1st time like a pro!

Alex fully concentrating on his bike without training wheels. yay!

We're only into the 3rd week of summer but the boys are developing a good routine to keep themselves busy throughout the day. When it's time for the babies to get therapy, they go upstairs to play video games or go into my room to watch cartoons (if not they'll stand over the therapist shoulders and be like protective body guards anytime they hear the babies cry) so that the therapist can do their jobs without having to answer to Ben and Alex. :)

For Father's Day the boys made him colorful cards and we all stayed out of his way while Chris was busy all day with cleaning out his closets and organizing things. I told him he is supposed to be lazy on Fathers Day (since I don't lift a finger on Mother's Day!), but he had the urge to purge so we let him go after it. It was a very low key day for him here at home. Then this weekend we celebrated his birthday. Viktoria made him a surprise German chocolate cake. Unfortunately the cake deflated so it really looked like the Grand Canyon but it tasted so scrumptious it didn't matter what it looked like since we all covered our pieces in ice cream. Ha!! Then Saturday night we went out for drinks in downtown then up to Allen to a Brazilian steakhouse his friend owns. It was a weekend finished up with playing around in the pool with the boys last night.

Ben and Alex are staying busy with a 2nd grade curriculum book. Chris worked out the lesson plans so we sit during the day and do the daily lessons. Ben's lesson on Friday included to compose a "poem" about someone. He chose to do it about Chris. He was hard at work and wouldn't show me anything til he was finished. Half way through the poem composition, Ben looks up and says "Mami, what's a word that rhymes with bald?"

For Father's Day I had the boys make a magic time machine card. On one side of the card they drew what their favorite thing to do with Chris is (in the "present"). Then on the other side they drew what their favorite thing will be to do with him in the "future". Alex drew shopping with him in the present, and playing poker with him in the future. Ben drew playing basketball with him in the present, and playing with his kids & Chris in the future.

Today after Chris told the boys he was going to teach them how to ride a bike, Alex said "Hold on, let me get a first aid kit put together!" To our grand surprise, once the training wheels were taken off, both boys zipped on their bikes as if they had ridden for years. It was nuts! No need for bandaids. NICE!

Dorian is working on making the "SH" sound with his speech therapist. He can't work it out with his mouth, so he just blows out of his nose when he is prompted to do it. It's so cute. She loves it when he does it. Wynn is trying his best to mimick facial expressions and sounds. He loves to roll up to Dorian and try to hold his hand or touch his face but Dori gives him a swift kick or a loud yell and Wynn knows to back off. It's hard being such a snuggle bug if your bro doesn't want to snuggle back!! We need to send Dorian to some sensitivity training. :)

This week the babies have both been party boys in the middle of the night. They get the night nurse to feed them then play with them for at least an hour, or FOUR. They keep the babies happy so we don't hear any fussing or crying to actually wake US up. Twice this week Dori partied til 4 or 5 AM. Hopefully this is just a phase, but at least while they are awake with the nurse they are happy just talking and playing with toys. Maybe they are practicing for college all-nighters (?).

Mavs Maniac

2011-06-16T18:00:00.000-07:00

Jason the Jet Terry & Dirk

Dirk doing the Dirky

Tyson Chandler

The awesome parade crowd!

My two cute parade dates!

I'm happy to report that in our household we have come down with a serious outbreak --- of mavs fever!

What a fun past week it has been... staying up late with the boys to watch the games, trying not to cheer too loud because loud cheers scare Dori, being proud of Wynnie staying up real late with us to join in on the chanting, doing victory dances for our night nurses (who I have no doubt think we are totally nuts), and just sharing the excitement of the mavs NBA championship with Ben and Alex. It's really neat to see their excitement. Chris says I'm a "superfan" and Ben is not far behind from me, he likes to watch the games over and over again.

Today we woke up at 5:15 AM and took the train to downtown... sunscreen, DS's, sunglasses and cooler in hand (packed with sandwiches and gatorades). We left the babies at home with Viktoria because I thought the crowds and the heat would be too much for them... that was the best call I made all day since there were over 200,000 fans and over 100 degree heat.

Ben LOVED the train ride and was so pumped to see houses and businesses all dark as we choo-choo'd all the way to Victory Plaza, he kept clapping and saying what a good day it was. Alex within 5 minutes of boarding the train Alex said "This is my second most important dream come true!" I was so excited that I didn't even think of asking him what his FIRST most important dream is. I just gave him a big hug. Since we got there 4 hours before the parade started, we got a great area to watch the parade from... here are the pictures. What a fun time, I'm so glad I was able to share this experience with the boys. CHris wasn't able to take off from work to join us but we kept him informed through phone calls and texts.

Thanks mavs for giving our family so many neat memories this year!! As I screamed to Dirk, Terry and Kidd as their float went by "WE LOVE YOU GUYS!!!!"

Let's go Mavs!!

2011-06-05T16:18:00.001-07:00

Game 3... tonight here in town... we've got Mavs fever, and only a NBA championship can cure it!! I made the babies' mavs shirts last night (iron on letters) since no stores carry Mavs gear so small. The boys are really excited... Let's go Mavs!

New bath chair & pool time

2011-06-02T14:53:00.001-07:00

The babies just LOVED the water

Dori's new bath chair... what a handsome fello!

I couldn't wait to post this and share with you all... Dori and Wynnie apparently have a new passion -- swimming! Well, more like hanging out in the water with cute German girls. What guy doesn't?

Here is a picture of them with Viktoria and her sister, Olga. Sadly Olga is leaving tomorrow after visiting for 6 weeks. We know Viktoria will miss her big sister, they've had alot of fun these past couple of months.

Dori also got his new bath chair and we tried it out today. He cried the whole time, not liking it one bit... but I was able to distract him long enough to get a picture of him without him screaming. Way to go Dori!

For more updates on their PT and Speech therapy, please view the blog post from earlier today.

June 2nd

2011-06-01T09:08:00.000-07:00

Dori smiling at dinner table

Wynn unimpressed by the baby dinosaur he was petting on Monday

Alex in field day

Ben with the look of a crazy man at tug-a-war (he gets that from Chris!)

School is over on June 9th so just a few more days and *freedom*... so that means lots of activities to plan and fun play dates to book so the boys have a fun and eventful summer. That also means Dorian and Wynn will feel like Atilla the Hun has invaded their private quiet days until school starts up again! ha ha ha!!

Just wanted to post a couple of pictures of the boys and babies lately... I tried many times to post new videos to this blog entry but for some reason it's not working. I'll post it once I get it figured out. It was some cute ones of the babies laughing, talking to me and eating my iPhone (otter box) case.

Football has had us really busy (the season ended last Saturday, they got 2nd place in the league). Now we're in full basketball mode. Chris started two select basketball teams and is coaching those. Our team name is "Big Red". No surprise there, right?

Wynnie has had a fever the past couple of days and throwing up, but I'm taking him to the doctor to see what's going on. Probably an ear infection I assume, but I don't know what his vomiting is about.

Dori has been improving by leaps and bounds in PT. With the help of his bobby pillow around his hips, he is able to sit up for a total of 7 minutes!!! His PT is really proud of him, as are we. He is also stealing the show at speech therapy. His speech therapist Hope has become a stay at home mom so we miss her dearly. :( But his new speech therapist Lauren is great and the babies have really blossomed with her. Dori is mimicking alot of her words during therapy and Wynnie is trying his best too. He recently discovered his tongue so he is more preoccupied with folding it over and sticking it out than actually saying words... but that's ok. It's a start!

Their old OT left the company so they have a new OT. The babies are taking a little while to get used to her but she does all sorts of fun things (play with shaving cream, color with markers) so they are beginning to warm up to her because of her new tricks and toys.

Wynnie is still everyone's friend... anyone willing to hold and cuddle him is alright in his book. As for Dori, he is developing his "stranger anxiety" like a typical 2 year old normally does. If he doesn't know you very well and you try to touch him, he'll let you know he wants you to step away... far away, fast. Lauren told us that is a good sign of his mental development since a "normal" 2 year old would also begin to exhibit that same aversion to strangers as well. So please don't take offense... it's just Dori being a normal 2 year old.

Ben and Alex had field day at school last week... it was a hoot! The babies enjoyed it for about an hour then they started getting hot and fussy (they get hot and sweaty way faster than normal since they are both on heart meds and that is a side effect). But before we left I was able to get some good pics of the boys in action. I was proud of them! The boys were very busy the last two weeks keeping watch of a mama bird that made her nest on our garage door opener. They rigged a step ladder so they could climb up and see the eggs and babies every day.

Chris made an observation last week that really struck me. This past year in Dallas we have hosted the World Series, the Super Bowl and now the NBA finals! Even though we haven't purchased actual tickets to go to any of those big time games, we've enjoyed the festivities around the city with Ben and Alex and it's been really neat to get caught up in all the excitement. Not many kids can say they've had fun at all those events, so it's a blessing to make those memories with them and our friends down here.

We hope you start to look at your calendars and think of when you might come down to visit us. The pool is ready, the lounge chairs are waiting and I'm sure we can whip up a nice cold beverage for you upon your arrival. Just to tempt you I'm putting picture of our front door and back yard on here to get your road trip juices flowing. Come on down!

Oh ... and one more thing... LET'S GO MAVS!!!!!

April 2011-Almost Easter

2011-04-18T20:53:00.000-07:00

Wynnie doesn't know a camera he hasn't liked!

Dori all smiles after breakfast today

Alex after school today at the neighborhood shops

Ben happy after eating a donut after school at the shops

It's less than a week away from Easter and we are looking forward to spending some downtime together in the house this upcoming weekend. The babies had a follow-up appointment with their cardiologist and the ENT. Thankfully, there is nothing to report! Their hearts are in the same condition they were 7 months ago, so for us that is great news. Same with their ENT, he didn't see anything different than last time with the little he can decipher from their extremely narrow ear canals. (no news is always good news in Dori and Wynnie's case!)

The ISMRD, an internationally focused nonprofit that advocates for families and patients affected Glycoprotein Storage Diseases asked us to write an article about Dorian and Wynn's condition and how we manage the juggle for their upcoming newsletter. The article ended up focusing on the lessons the babies have taught us. Take a read if you'd like, I've copied/pasted it into the body of this entry:

* * *

“How old are your babies?”

“Oh wow, they are so small!”

Those are things we hear a lot around here. I’m the proud mom of four energy filled boys. Ben and Alex, who our 7 year old twins that play every sport under the sun; and Dorian and Wynn, our 2.5 year old twin boys that were born with Mucolipidosis II and have taught us so much in the 2.5 years they have been here.

After parenting two perfectly healthy boys, when my husband Chris and I found out we were expecting another set of twins we were beyond surprised. We thought “will another set of twins stretch our time and patience? Will we be able to provide the same amount of loving time with the older boys with two new babies needing our care and attention?”

We went full steam ahead with this new adventure. The older boys took much pride in helping set up the baby room, putting the old cribs together, and picking out names. They took a lot of pride in knowing that being an older brother came with a lot of responsibilities, and they were excited for the big day of their baby brothers’ birth.

After six weeks in the NICU, the doctors diagnosed the babies with ML2. It was devastating to hear that Dorian and Wynn’s life span would be very limited. That they would only live to maybe 5, maybe 6 years old. How do you tell a parent that their baby comes with an expiration date? Although the news has hit us very hard, we are blessed to have a wonderful team of caring doctors, a supportive family, and an army of loving friends.

I am happy to say that Ben and Alex have taken on the roles of protective big brothers to heart. Alex is always showering Dorian and Wynn with all kinds of love and affection. Ben is the watch dog of the family. If he thinks a nurse or a therapist isn’t doing things right, he’ll be the first to speak up and protect his baby brothers in an instant. It melts our hearts when we watch how much they love their brothers. We have intentionally not told Ben and Alex that the babies have a terminal illness, because we want them to focus on having fun with them today, not worrying about what may/may not happen tomorrow.

Dorian and Wynn are only 2.5 years old, but they teach us important life lessons each and every day. Here is just a few of the things they have taught us already:

1. Don’t take a single day for granted. Even if it seems like the day has shaped up to be a “bad” one, there is always room to find blessings in each day and time to turn it into a “Good Day!” as Dori loves to say.

2. God’s grace and love for us is shown in many ways – a hug from a neighbor, a smile on a baby, a good report from a doctor, a new flower blooming in the garden. We have to keep our hearts open to actually see and appreciate these little gifts because it’s the good feelings that comes with those gifts that will be engraved in our hearts forever.

3. Big dreams and little dreams are equally powerful. Before the babies were born we had big dreams for them. Maybe they’d be life-saving doctors, maybe they’d be star athletes, maybe they would be the first twins to be President and Vice President! Who knows?! But we learned that you don’t throw away your dreams for your child just because they were born with extreme special needs. You learn to alter your dreams to suit them, and you celebrate each and every little milestone along the way.

4. Don’t focus on the negatives. You can’t change what has already happened, but you can always change the attitude you use to overcome what has happened. Whether you realize it or not, we are all surrounded by negativity. Negative reports on the news, negative family members, negative co-workers can all form a haze of negativity around you. What you choose to internalize is your choice. Negativity is like food – the more junk food you eat the more it deteriorates your body. Negativity deteriorates the soul, and in our home we choose to keep negativity at the door.

5. Families are amazingly powerful. Dorian and Wynn have helped us create a beautiful family of friends, doctors, a team of therapists (PT, OT, Speech, Auditory), night nurses, sitters, neighbors, friends and loved ones. Family are the people you choose to let into your life, the people you choose to confide in, the people you would do anything for. Dorian and Wynn have strengthened our family beyond anything I could have ever imagined. We are so blessed.

God did not give us more than we can handle. We feel so fortunate that God chose us to be Dorian and Wynn’s mommy and daddy. Those little stinkers will continue teaching us the power of love, patience, faith and family. I only hope they can help change your perspective too. Many hugs and blessings from our family to yours.

* * *

No Foolin, it's April Already!

2011-04-02T13:59:00.000-07:00

Ben with his masterpiece, "Ben Golden Adventure"

Alex with his masterpiece, "The Amazing Case"

Wynnie enjoying his 1st football game of the season

Proof of Dori's bullying ways!

This was "test" week for the babies. They had their OT, PT and Speech evaluation reviews. They have to do reviews every 6 months to prove to insurance that the babies are progressing enough and needing still therapy enough to justify therapy coverage. Plus, it's important for us as a team (we as parents and the therapists) to reset our goals for what milestones we want them to achieve for the next round of reviews.

For speech, the Dori has proven to be teachers pet! He has the verbal and communication understanding of a 1-1.5 year old. Go Dori! The therapist did note that when he wants to responds but doesn't know what to say, he falls back to his favorite word of "Hi". That's ok, I say "uh" to fill in the silence sometimes so that is fine. :)

Wynnie is catching up now that he more consistently wears his hearing aids. He is trying really hard to physically imitate when people are gesturing and talking around him and his aggressive at trying to get your attention if he thinks your not focusing on him. Although his verbal isn't as advanced as Dori, he is still a man with a message. He ranks around the communication skill of a 6 month old. Better than last time so we're happy.

For occupational therapy (hands and upper body stuff) Dori is around a 9 month old. We are challenging him more with grabbing his own toys and reaching for what he wants. Wynnie is around a 6 month old.

For physical therapy (lower body and sitting), the categories are graded pretty "hard", I asked them if they could score the babies on the curve. NOPE! Dori can sit independently, but it doesn't count because he can only do it for a minute or two and with a boppy around him, so he scored a zero on sitting. But even though the graded them pretty tough, Dori's leg and back stability graded him around a 5-6 month old, and Wynnie was around a 4 month old. We need to get him to have better head control before he can move onto more complicated body functionality. He gets his big nugget from Ben :)

The babies saw their pulmonologist and he was so happy to see how good the babies are and that they are breathing so well and have had a relatively perfect winter (respiratory wise). Yay!!

We are so proud of Ben and Alex. They had a author's reception for the books they wrote. Ben dedicated his book to his family, Alex dedicated his book to his class. Both boys got on stage to say that so we practiced and practiced. They spoke so confidently!!! Ben even crossed his arms like he was rappin it. So cute! Professional speakers of the future??

Alex is a flag grabbin fool at football... he is a BEAST!! Ben is trying to find his groove on the line and as the season progresses we know he'll be back to being a major force on that line. They love their team and so far we are 1-1. Go Bears!

Our hearts are very heavy for lil Ethan from Minnesota. He was another i-cell baby who was hospitalized a few days ago for RSV. He was having a very hard time breathing. His mommy whispered in his ear that he could go and within the hour he passed away in his parents arms this morning. The news hit me like a truck... our prayers and love are with his parents and extended family. I'm so sad to know that people have to go through this, I hope they have a good support system to help them through the days ahead. In the meantime, we'll keep spoiling our babies rotten, and taking them everywhere we can so they can enjoy everything life has to offer.

Check out this video... it's Ben doing his nightly ball handling drills with Chris. Ben didn't know I was recording him from my laptop in my office. This little kid is so determined, his gumption always inspires me!

Random Observations & Book Recommendations

2011-03-21T20:57:00.000-07:00

Ben took this picture while he was making Dori laugh. Our good camera broke so all we got is the iPhone for now His image looks like he's at a weird mirror in a carnival fun house, right?

It was a gorgeous day at our neighorhood farmers market and lake, but Wynnie wasn't in the mood.

The boys in their smarty pants shirts I got them in Boston. I also got to see my baby cousin who is a freshman at MIT.

The boys in the backyard on Sunday

Major victory this weekend!

100% Benjamin. David. Johnson.

My new friends and their sweet baby Amber from Boston!

Hello all! Today I figured I'd capture some observations that I wanted put down so I wouldn't forget. Don't try to follow any logic or a path in this entry, this is my mind free-basing tonight.

1) Dori is in his crib right now (nearly 11 PM) and is saying "Good day!" "Hi!" and howling like a werewolf. He's an interesting little fella. Last night he partied in there til 2 AM on his own while the nurse listened on.

2) Wynnie had his ortho boots on yesterday and he rolled rolled rolled til he could get to Ben (who was sitting on the ottoman playing his free skateboard apps on the iPad). When he got to Ben he kicked him over and over again to get his attention. All I heard was "Geesh! Stop it Wynniiiiie!" I told Ben "You know when you mess with Alex and I tell him to not let you get into his head? Well... now it's your turn. Don't let Wynnie get into your head." He got up and moved to the sofa. It was fun for Wynnie while it lasted. Dori must be teaching him a thing or two.

3). A new book I can't wait to download is "Heaven is for Real" by Todd Burpo. It is simply amazing and beautiful to hear of this 4 year old boy from Nebraska having this experience. Read this article, I bet it'll make you want to get the book too. When adults write about heaven or near death experiences, I get cynical sometimes. Sounds bad I know. But when it's from a 4 year old boy who's parents at first didn't even believe him (his dad is even a pastor!), it really got to me. http://www.nytimes.com/2011/03/12/books/heaven-is-for-real-is-publishing-phenomenon.html?_r=1&src=me&ref=books

4) Alex and Ben came home from school talking about how they helped people, even when they didn't get anything back for it (we're trying to get them to do nice random things for people during Lent). I was so proud. Then two hours later, Alex tried negotiating with me if he could have $1 for every dirty sock misplaced around the house. I said no deal. Then he asked if him and Ben could have a garage sale on Saturday cuz he has a list of toys he wants to buy with new money. I said no to that too. Gotta love the entrepreneurial spirit in that sweet boy.

5) I was gone Saturday - Thursday night working in Boston so I missed the boys, the babies and Chris so much. Unfortunately Chris got sick, the babies were both sick, Viktoria got sick, I had bronchitis the whole time I was there (I still do, but I'm on the mend). Dori is nearly back to normal, but Wynnie is regressing with acting really quiet and lethargic, and I think he has pink eye too. Chris is still sick. Looks like we need to get ourselves our own carport at the doctor's office. But I think we'll all be getting better here in a few days.

5.5) While in Boston I had the honor of meeting Amber, an 18 month old sweet little girl with ML2. I've connected with her parents online through an ML2 parent support group and when I mentioned I was headed to Boston on business it was perfect timing that Wednesday (my free day where I had zero presentations to deliver) Amber's parents were driving in from their hometown 2 hours away from Boston to go to Boston Children's for Amber's metabolic geneticist appointment! yay!! My most favorite part of that whole trip was meeting precious little Amber, and meeting her kind parents. I was so blessed to meet them, I cried when I had to say good-bye... they seem like really lovely people. Amber wears cute little glasses, has long voluptuous hair, and sports a little oxygen tube in her nose. I just wanted to give her lots of hugs and kisses but I was sick so I didn't want to infect her. Since she's from the east coast, she totally says her name with that east cost accent... her daddy pointed to her and said "Who is this?" and she said "Ambuh". I loved it!!!!

6) Alex FINALLY lost his front tooth that had been loose since mid-December. It was actually starting to turn a weird color so I think the tooth was starting to "die" or something. So we tied tooth floss around it and had Dori yank the string. Dori gave it a nice tug but the whole string came off. Then Ben wanted to pull it out for Alex but he is not very "soft handed" so he got turned down on that offer. So then I finally yanked it out. Alex was so excited he said "This calls for a celebration!" ... so had me put both babies on the floor, blast on the music and have a dance party. It was cute.

7) Our friend Mark that lives in Louisiana is known as "nino Mark" to the boys. After we prayed for the tsunami victims Sunday after church, Alex said "I'm sure glad nino Mark didn't die in the tsumani!!" And I told him Mark was safe cuz he lives in Louisiana, not Japan. Well I guess Alex thinks Louisiana is as foreign and far east as you can get if he thought Mark got swept up in all that stuff. Mark got a good laugh at that.

8) Another good book is "The Help".

9) When I go out with the babies, if people ask "How old are they?", when I say "Two" they either look at the babies funny like "hmmm..... I'm no pediatrician but that baby sure look tiny for a two year old" Sometimes they go as far as saying "He's so small!" so I just say "Yep, he likes to grow at his own pace. He's in no hurry." OR if it's one of the nurses at one of the offices we go they nicely say "WOW! They are getting SO big!!" I don't know which is better. The nice lie that we both know is just kind words, or the honest funny look. If I was on the other side of the fence, I might blurt out "oh wow, he's so tiny!" so I really can't judge.

10) I've found alot of inspiration from other ML2 families on what they are going through, what advice their doctors are giving them and what good attitudes they all have about their children, and their struggles. I wish I could win the lotto and give a million dollars to each of them - so they can all quit their jobs and spend all the time in the world they want with the precious little angels. Each family has such a story, no one has it easy. I'm so thankful that Dorian and Wynn have done relatively well over these past 2 years... a few hiccups here and there. But all things considered, the babies have been doing so well and I can't believe two years have already flown by. I want time to slow down for just a little. Not enough time... but I guess that's the case for all of us no matter what.

11). Since Chris is sick in bed I had no one to ramble on and on to this late at night, I thank you for indulging me. Speaking of rambling, Dori is incorporating his rattle into his repertoire now and keeps saying "YAY!". It's 47 minutes since I started writing this and he's still going strong. I can only imagine that Wynnie blocks him out well enough to have dreams of dancing at Studio 54 and Dorian is performing on stage. Rock on babies!

March 2011

2011-03-01T11:16:00.000-08:00

Dori overseeing homework at the kitchen table

Alex and I during his Valentine's Day party at school

Us at the long term care facility after we delivered a big laundry basket of treats.

Cheetos and other snacks for the kiddos at Children's

I'm sitting here watching the babies get their speech therapy and it's enlightening/ entertaining to hear. Our ST is going on maternity leave real soon so she has her replacement here for training, so it's been neat to hear her debrief the new girl. Dori has full grasp of 8 words and prefers to call Ben "die die". We weren't trying to make a big deal in front of Ben (acting like "oh no, I don't think said that, it just sounded like it") but Ben over the past few months Ben is like "See!! He called me Die Die again! Ugh! Why does he call me Die Die!?!?!" Even during therapy when Ben's face is shown to him with the 8x10 flashcards, he is consistent with calling him that. Poor Ben, we need to get Dori to call him BEN! I can't help but think it's a little funny.

Wynnie was a show-off all smiles and cuddly with the new lady. He's trying to get on her good side. Ever since he's been wearing his hearing aids since January on a more regular basis, his interaction and responsiveness has shot through the roof. He's back to his ol babbling and expressive self, so that's so nice to see.

Basketball is over for Ben and Alex. Alex did a great job of learning the game and really liking it this year. Ben is so good at making baskets. He'd be pulled out of the game after only 2 quarters with over 20+ points to let the other kids play. I'm so proud when they play so well, especially since they enjoy it so much.

This weekend we took alot of treats to a long term care facility in Plano. In honor of Gabriella Scarpelli (a sweet little i-cell baby that earned her angel wings a year ago this weekend). Her mother shared that Gabriella loved to make people smile. While they spent alot of time at the Ronald McDonald House while Gabbie got her bone marrow transplant, they would receive alot of snacks and food gifts from strangers and it really touched them when strangers out of nowhere would brighten their day with their time and their gifts and thoughtfulness. So in memory of Gabbie, her mom wanted as many friends and family to help put smiles on the faces of people going through health difficulties. Then yesterday we went to Children's Medical Center with Viktoria and the babies to drop off a wagon load of cheetos and other snacks for the oncology kids. When those kiddos are going through radiation, everything tastes like metal. Cheetos is one of the few things that they can actually tastes through all that metal aftertaste. Hence, the wagonload of cheetos. The babies had fun driving all the way to downtown to make the delivery.

The boys and I read 4 bible stories the other night, then they got to pick their own books to read while I fed the babies. Alex chose a Star Wars book and after thumbing through it said "The bible is like Star Wars and God is like the Force." Pretty awesome.

For He's a jolly good DORI!!!!! Nobody can deny...

2011-02-04T09:08:00.000-08:00

Ice all week, now it's covered by beautiful snow

After 24 hours stuck at home, we finally ventured out for a lunch date. Yum!

Dori was in a party mood with me, not wanting to sleep the other night

This is blurry cuz Wynnie kept moving so fast I couldn't get a good snap!

I am happy to announce the Dorian Allen Johnson has been awarded the Standing Ovation Award by the National MPS Society. This award is given to a brave boy or girl who shows courage and inspires those around him in his battle against the family of MPS related diseases.

We are so proud of Dori!! We haven't told Wynn because we don't want him to get jealous, so we are keeping the celebrations low key. I've been sent a questionnaire by the MPS Society asking for Dori's bio so they can do a write up about him... so it'll be fun writing about his "likes" and "hobbies". Go Dori!!!! When they called to tell me this week, I was a big old ball of pride... it's heart warming to see him win an award for something, especially something that means so much for what he and Wynnie go through on a daily basis. They are so awesome.

We are on day #4 of school being cancelled due to major ice and now 6 inches of snow. Yesterday we had a total of 7 boys at the house playing... cabin fever has turned out fun for hte boys - lots of friends over and lots of video games. This weekend is superbowl just 45 minutes away from our house, hopefully everyone will still have a nice time despite the ice.

Wynnie's ears are all better and we FINALLY found his hearing aids that had been lost since October. Now that he's wearing his aids, he's alot more vocal and interactive. The old Wynnie is back. Watch out ladies! Wynnie is really good at skyping with Viktoria's family and friends in Germany. He is creating quite the following. Maybe he'll be the next David Hassolhoff.

Dori is a jabbering social bug, he makes us laugh alot. He's great at blowing kisses and is still all about calling out for me. He has fun watching Ben and Alex wrestle, and he tries to follow your tune when you sign to him. His favorite thing to do while watching his YouTube videos is to watch YOU while you sing along to the videos. He likes the double entertainment.

Happy New Year 1/15/11

2011-01-14T21:57:00.000-08:00

Happy New Year! Dori and Wynn had fun ringing in the new year.. they didn't make it til midnight but they sure tried. Ben and Alex on the other hand were wired until 1:30 AM!

Dori has become quite the instigator to poor Wynnie. He loves to roll around on the floor and chase him. When he "catches" him he kicks Wynnie, or tries to grab his head. We've had discussions at length with Dori about the perils of bullying but he doesn't seem to think what he does is bullying. Wynnie sure does... he lets out a loud cry when Dori gets him. Even Ben has jumped into action by creating a toy barricade between the babies. I love how protective Ben is of his baby brothers.

Speaking of Ben, he was jumping on his bed past bedtime and cracked open his head (hitting it on the bunk bed). Chris took him to the ER and got him 2 stitches. He got his stitches out yesterday and was informed by our pediatrician that there's a Stitches Fairy. She is the Tooth Fairy's sister. So when they got home, Alex bartered some candy for one of Ben's stitches so that both he and Ben could score some cash from the Stitches Fairy. Unfortunately, Chris and I fell asleep and we forgot to put money under their pillows. I should invoice our pediatrician for bringing it up. ha ha!

Wynnie is a super cuddle bug with Miss Viktoria. Dori has a new-found infatuation with me, always calling for me, always has to keep his eye on me at all times. I guess my business travels have made him realize how much he LOOOOOOVES me :). But he drops me like a sack of potatoes when his night nurse comes cuz he knows he gets to watch his YouTube videos with her for as long as he wants.

Alex told us today at breakfast that when he gets married he'll live in this house with us in it. I keep telling him that when he's married he'll probably only want us to visit, not to live with him all the time (he brings it up at least once every 4-6 months). I promised I'd watch his kids for him while he goes on dates with his wife and Ben said "papi too! papi gets to watch the babies too!" Then completely out of character, Ben told Viktoria he liked her and that he wanted her to "stay." When she leaves he is always saying "Why does she go out with her friends all the time?" Can we say... crush?

Chris is doing great... moved his office back to the Dallas headquarters so he's getting back into the groove down there. He's been busy with work and helping the boys with their basketball practices. Ben's a good shooter, but Alex is a spirited defender. It's so neat to watch. Chris is planning a Texas Hold Em style poker event to raise money for MPS society. We're still trying to nail down the dates, but probably in mid-late March. Stay tuned!!

Ear Surgery and radio stars

2010-12-09T14:43:00.000-08:00

Me in Dubai. The boys look cute in the jerseys I brought home for them.

Ben and Alex posing this afternoon with Gingerbread Fred in front of the tree.

Wynnie playing "peek a boo" with Papi while waiting to get his tubes today

Dori hanging out with Papi while Wynn was getting his tube in.

Babies were on the radio this morning during morning rush hour. woo hoo!! It was for the Children's Miracle Network radiothon, to raise money in the Dallas area. Hopefully their story will help much needed funds for CMN. Their story will continue being aired throughout the radiothon this week on Dallas's 103.7 Lite FM on the following dates/times:

Thursday, Dec. 9

8 a.m. and 4 p.m.

Friday, Dec. 10

10 a.m. and 2 p.m.

Saturday, Dec. 11

2 p.m.

Aside from being radio stars, they also went into surgery today for new tubes in their ears. They did great, relatively happy throughout the morning as they waited to go under. When Dori came out of anesthesia all groggy, he opened his eyes and blew Chris a kiss, then fell back asleep. So so sweet, he's our loverboy. Both babies were only able to get one ear operated on, because the doctor said he's never seen ear canals so narrow before, so he wasn't able to get in there.

Ben and Alex brought home Gingerman Fred from school. They are supposed to take pictures of their normal "happenings" with Fred tonight and send it back to school in the book to share with their classmates. Fred is pretty cute, the boys think it's so neat.

I was gone all last week in Dubai for work. It was very beautiful, very modern and very rich in cultural diversity. I had a ball shopping and everyone was extremely nice and helpful. The world's largest mall was across my hotel. I went for the gala for The Christina Nobel Children's Foundation, and the rugby tournament her foundation sponsored. It was a super fun time, but I really did miss the boys!! Alex was telling our night nurse that I was gone at Jumangi. I guess Dubai is so far that it sounds like it would border Jumangi. ha!

Wynn's attitude problem in PT

2010-11-19T08:26:00.001-08:00

This morning the OT and PT therapists were both here at the same time working on the babies. I have to post this picture... they put the babies face to face to see them interact. Dori loves his "lazy boy" bumbo but Wynnie was hating it today, so the whole time Dori is smiling and talking to Wynn and Wynnie is screaming mad!

Last night Chris had the babies on the floor and was pretending to "wrestle" with them. He put Wynn on his chest and was acting like Wynn was beating him up. Ben ran over and said with a really worried face "Papi are you hugging him your softest?" We had to explain to Ben that papi was only "pretending" to wrestle and that the babies were fine. That was so sweet of Ben!

Tonight Viktoria arrives ... it'll be so nice to have her here and not to mention all the help she'll provide to all of us.

Football, birthday party and fun! (11/15/10)

2010-11-15T16:41:00.000-08:00

Boom Boom Pow! Ben getting a flag at the All Star Game!

Alex in the middle with the running back's flag.... sweet!

Dori loves story time with his night nurse.

Don't let Wynn's face of horror fool you, he loves his birthday party. The theme for the party was "You are my sunshine" since they are that and more! Their shirts say "I will make a difference", so so true!

Dori having fun around the house with papi.

The babies are proving that terrible twos are just a myth.... both had a happy weekend relaxing at home playing with their brothers and spending quality time with papi :). We are anxiously awaiting the arrival of Viktoria (from Germany). Today she arrived to NY for her Au Pair school training, so by Friday night she'll be joining our family. Welcome to the circus! I mean, welcome to your new home!

Ben and Alex continue super busy with their football season. Since they were picked for the all star team, we continue with practice 3 nights a week. They won their 1st all star games in big fashion, so they'll be heading up against flag football teams from all over the US this upcoming weekend. How exciting! Our collection of trophies is getting shinier and bigger. They are so proud of themselves, as are we. The ultimate goal if they make it past all the games this weekend would be to play Super Bowl weekend amid the festivities at Cowboys stadium. One game at a time...

Our National MPS Society fundraiser was a total blast! I hope to get pictures from people soon, but we had alot of hilarious costumes and people with a fun and giving spirit. This year we doubled what we raised last year, so I was happy to send over $4,700 worth of checks to the good folks at the National MPS Society. We allocated the money that Dorian & Wynn raised to go 75% to research (we need to find a cure!), and 25% to go to family support (for other ml2 families that may have financial burdens from medical bills, or in need of home medical equipment that isn't covered by insurance). We are so humbled by the generosity of everyone that participated in the party by donating items for the silent auction, buying raffle tickets and just having a great time for a great cause. So grateful!! So happy!

Dorian and Wynn's head

2010-11-01T22:17:00.000-07:00

The babies had a busy day today. Wynn had an appointment with the dental surgeon for the big cyst he has growing on his bottom gums. After doing a little more research, he called the house and told us that he wouldn't recommend doing any surgery on it at this point. Which is great news because we don't want them to undergo any surgeries that are not absolutely necessary. So in the meantime, Wynnie will have a cyst the size of Mt Everest in his mouth but he doesn't seem to act too bothered with it so we are fine as long as he acts fine. Poor little guy!

Then we took the babies to Medical City really quick to get paperwork, then over to Children's for them to see the neurosurgeon Dr. Sacco. Posted above is Dori's cat scan from last week. The very noticable bump is on the back/bottom of his skull. You'll see a break/gap of skull bone underneath where the bump is. From what he saw in the scan and what he felt upon examining him, he thinks it's a dermoid cyst. Basically it's harmless. Unless the cyst begins to change alot or if he seems he is in pain, Sacco said no need for operating.

As of last week, Wynnie out of the blue had a bump pop up on the bottom of his skull too. So weird how a couple of weeks ago neither of them had anything and now within a week they both do(!!!). Since all of this sprung up not even 2 weeks ago, Dori's bump has gone from soft to hard.

Why does they have those bumps there? In a healthy baby, the skull bones are not fully formed and fused together, there are many gap areas in the baby skull at birth. As a child grows, so does the brain, so these gaps in the skull allow the brain to grow within a flexible area that allows for that growth. In Dori and Wynnie's case, their skull bones have nearly all fused together (far earlier than normal), so as they are getting older, their brain has no where to grow. So their heads are a little differently shaped, Wynn has slight bulging on the sides of his head above his ears and Dori head is uneven and pointy.

For both babies, the verdict is to do nothing. At this point it's not causing them any pain or visible discomfort so Dr Sacco felt that their cardiac and respiratory problems outweighed the risks that would be involved in operating... the only way to fix this would be to do cranial surgery to basically give the brain that room to grow (cutting through the skull to give the brain more space), so we agreed with the doctor that is completely unnecessary in the babies' case.

Chris and I were impressed that Dr Sacco really is famous. ha ha!! He and his team at Childrens were the ones that did the famous surgery on those two little boys from the middle east (conjoined twins that were attached to the head). They had a picture of the boys in the office so it was clear that Dr Sacco and his colleagues are top notch. woo hoo!

On a faaarrr more positive note, what a fun weekend! Our MPS Fundraiser party was super fun, we had a great turnout, lots of people donated for our raffle, and lots won some great prizes with the silent auction as well. We're still getting checks rolling in for the MPS Society so I won't know the grand total until we count it all up, but I think we doubled what we raised last year. woo hoo!! We're so thankful for the Caracalas's and the Mazurs for co-hosting this party with us, and for all our friends and family that showed up and had a great time. I went dressed as Tiger Woods, and Chris went as my lovely mistress.

Check out the Johnson boys in the costumes... Alex was a knight, Ben was a ninja, and the babies were pumpkins. How cute!! :)

Dori's nugget, part 2

2010-10-24T19:47:00.000-07:00

Dr Hermann, the pediatric surgeon, ordered an MRI and later a CAT scan for Dorian's growing lump issue. When we arrived to Medical City we couldn't do the MRI because the anesthesiologist (Dr. Kampine) told us Dori was too high of a risk to intubate & sedate during the procedure due to his major heart problems and constricted airways, so we did a CAT scan instead.

We could only get one shot at keeping him perfectly still for the images to capture. After going through ALL the verses of "Wheels on the Bus" and "Head, Shoulders, Knees and Toes" Chris and I were able to keep him entertained enough to do it without any setbacks.

Through the CAT scan Dr. Hermann found Dorian has abnormalities in his bone structure in his skull, a hole in his skull that is letting brain matter &/or cerebral fluid pop out of the hole. He told us this is beyond his expertise so now he has referred us onto a pediatric neurologist who can take a look at Dori's head and figure out what next steps should be (Dr. Sacco).

The good thing, Dori doesn't act like it hurts when you touch it so he's not in pain, but it may be causing him headaches from that pressure point. The anesthesiologist and his colleague (another doc he called in on her day off) were very candid about the permanent risks of putting Dori to sleep, and the possibility of cardiac failure if intubated through his narrow airways. Dr. Kampine is hands down the best pediatric anesthesiologist in Dallas... coming from a dad who literally wrote the book on anesthesiology (his doctor dad was a professor in med school to aspiring anesthesiologists) so we fully trust his concerns. Hopefully we'll get into see the neuro guy asap, don't know how far out since these guys calendars get booked pretty far out.

Just like in any profession, you are going to encounter people that are really "good" at what they do and people who are just average. We've been very best to be connected to very very good doctors throughout this process so that makes things alot easier when you have full confidence in their medical opinion.

Ben and Alex have been entertaining the babies much to their delight. Dori laughs out loud with Ben's antics, and Wynnie flaps his arms and kicks alot when Alex comes over to play. Wynnie figured out that as long as he doesn't cry, he can hang out as long as he wants to in our bed while they watch cartoons.

Just 3 short days til the babies turn 2 years old!! We are so proud of them!

October 2010

2010-10-16T20:27:00.000-07:00

This is the best... Wynnie is world's best snuggle bug

Winner Winner Chicken Dinner~~

Kept telling Alex "smile like you're NOT pooping" and this was the best I could get

Dori pondering over the Husker stats with Chris... hmmm....

I'm so proud of all my boys! Ben and Alex are really taking an interest in reading. Report cards came home and Ben showed us his ribbon for making honor roll. We jumped up and down and gave him a big hug and Alex was really quiet. We could tell he was sad, so we said "That's ok Alex, we'll get it next time right?" Then he walked on over to Ben, patted him on the back and said "Congratulations Ben." It was so sincere I almost cried! Then Chris took Alex's backpack, turned it upside down and shook it, out fell Alex's honor roll ribbon. When Alex saw his ribbon he lit up! He was so excited he started screaming "I made the honor roll! I made it!" and he took the ribbon and hugged it. I almost cried again! It was so awesome!

The Colts (Ben and Alex's flag football team) continue to be undefeated and next weekend they will play for the city championship. Chris has fun coaching them and they are on a team with alot of sweet kids.

The babies are so full of personality. When Dori gets mad at his nurses he screams "ay ya ya ya yaaiiiiieeee" over and over again until they give him what he wants. It's so funny because I haven't noticed him doing that to Chris or I yet, only the nurses. He just started doing that 2 nights ago. He's connected with his Latin side, speaking his mind in Spanish of course. :)

Wynn loves people! Today 2 neighbor girls came over to look at our halloween decorations and Wynnie was smiling and cooing and trying to touch their faces the whole time the little girls were here. It was so cute. In the background, Dori just kept saying "Hi! Hi!! HIII!!!!".

During our parent/teacher conference at school, Ben's teacher told us how proud Ben is of his baby brothers. She said he talks about them all the time. Then Alex's teacher told us how caring Alex is towards his classmates and what a sweet boy he is. It made us so proud! It's a good thing they don't see them when they are trying to scratch each other's eyes out at home over their DS's. ha ha!

The Au Pair agency got Aury matched with 3 other families (one in Chicago, one in California, and one in Pittsburg) but she turned them all down because she didn't want to leave Dallas. So instead, the agency sent her back to Colombia. In the meantime, Anna helped me all the way from the Ukraine to help me interview an au pair candidate that we were veeerrry interested in named Viktoria. Viktoria was born in Russia but her parents and her immigrated to Germany many years ago. She is a certified "caretaker" (not a nurse, but a certified care provider) and has experience with special needs kids. Anna wrote her a letter about our family and about the babies, and then Viktoria's mom called Anna to ask her honest opinion about her life with us and the demands of working for a family with 4 kids. Anna helped seal the deal, Viktoria accepted and hopefully we'll have her by Thanksgiving here in our home. We're so relieved!

Chris and I decided to take a last minute getaway to Las Vegas with our friends the Sundells. It was the 1st time we were both away from all 4 boys at the same time. We were gone for 3 days, but it was a nice little break from reality and relaxing to spend time with good friends. Marcia stayed at our house and took care of all 4 boys, and our friends the Mazurs helped entertain Ben and Alex by taking them to the state fair when they had Monday off from school while we were gone. I went to the Wynn casino to buy Wynnie a shirt and Chris a hat that say "Wynn" on it, and while I was there I thought "I'll play a little roulette in honor of Wynnie". Well I won't use this blog time to brag about how I was the HUGE winner (turning $40 into nearly $900) because this is not the forum for that, but I'll just put it out there in case anyone wondered. I think it was Wynnie's divine influence over my chips that day. hee hee hee!

Anyhow, the babies will get new tubes in their ears in November right before Thanksgiving, so hopefully that'll help with Wynn's ear drainage and discomfort problems. Today was a fun day with all the boys and I wanted to express how much love they give us by just being themselves. I took some time to video the babies playing with each other and talking to me today. We need to do more of that. We are truly blessed, and looking forward to the end of the month (babies' 2nd birthday and our MPS Society fundraiser party). If you are interested in donating, please contact me!

Fall, Fun and Fabulous Evaluations!

2010-09-30T08:08:00.000-07:00

Wynn taking control of his own heart check-up, grabbing the probe with a smile of course!

Dori Snuggling with Miss Jen (Bishop), future mother in law of Ben or Alex.

The UNDEFEATED Colts!! Alex 1st row, 2nd to the right; Ben 2nd row 2nd to the left

Wynn enjoying his breathing treatment

It's fall, and it finally feels like fall! It's only making it to the 80's so that's a nice cold front for us. Football for Ben and Alex is going great --- they are still undefeated in their final year of flag football (next year they move up to tackle). Yesterday the boys started their after school golf program... I think they like it. The boys have been doing great at school and they begin bringing homework and studying for spelling tests next week after report cards come out.

The babies have had their OT and PT evaluations done, and I'm happy to say both babies have progressed very well compared to where they started. In PT, Dori has advanced to the strength and mobility of a 6-9 month old, and in some cases 12 month old for certain skills. He is using little help in sitting up and he actually sat up for 3-4 seconds all by himself yesterday. The therapist and I started screaming happy but unfortunately we scared the crap out of Dori with all the hollering. oops! He started off with the ability of a 3-6 month old so we are proud of how much he has accomplished since the end of May when they started with their new therapy ladies.

For Wynn in PT, he has also made good progress as well. He started around the same level as Dori and he is at a solid 6-9 months, but lagging a little bit behind in Dori in many areas but that's ok.

In OT, Dori he is doing well. He's progressed to around a 6 month old (starting at a 3-6 month level). Wynn has progressed to around a 6 month old as well (starting off at 1-3 month level in most skills).

Yesterday they also saw their cardiologist (their new one since their old one retired). They after running tests and doing echos/sonograms to see their cardiac function the doctor bumped up their meds by quite a bit. Wynn's heart condition is basically the same as his last check up, but Dori's has gotten a little bit worse. His heart is having to work too hard so his left ventricle has enlarged (just like a muscle, if you pump your left arm with heavier weights than your right arm, then your left arm will be bigger since it's being worked harder). Enlarged hearts are a norm in ML2 kids so it's an expected progressive situation that we'll continue to monitor. They'll see their cardiologist again in 4 months. We'll continue to treat it through medicine than doing something more invasive at this point. It wasn't good news but he's as happy as could be so he doesn't know the difference. As long as he's not distressed and in pain, there's no sense in us getting worked up about it either.... just keep pluggin away.

We've been eating in our backyard and watching lots of football now that it's cooler weather. The babies enjoy being out there, Ben and Alex love having friends out there watching TV or playing around, and Chris loves his TV out there. And I can actually still watch my cooking/decorating shows during football games inside and watch the mayhem outside from my chair :)

The MPS Society Fundraiser that is coming up at the end of October is in full prep mode... Alma and Stephanie are getting the word out and evites will be going out soon. Start getting your costumes ready... bragging rights are at stake for the most creative, and prizes are being gathered for the raffle winners. yay!

Oh and on a final note, we fired our au pair. So Chris and I are reverting back to the olden days (before Anna came) when we juggle between work, big boy stuff and baby stuff. I'm actually having fun with the babies during the day so it's nice to spend more time with them. It didn't work out with Aury unfortunately... we gave it 4.5 months but she needs to be with a family that can better suit HER needs and take more time to attend to HER requests, so we're not the ones for her. We got spoiled with Anna who was very independent and very flexible with us, who was very fun and appreciative of things, so if we could find ourselves an Anna clone that would be PERFECT!! :) The agency is looking for a new au pair for us but since the babies are considered "special needs" it's hard to find a girl who wants to JUMP at the chance to be in a home with 4 boys, 2 that are special needs. Who wouldn't want to be a part of this rockin' bunch?! really!?! ha! Oh well... onward and upward!

Enjoy Fall y'all!

Buffalo Bills & Ironman (September 2010)

2010-09-21T19:30:00.000-07:00

What an inspired day I had today! I was proud of the babies getting their new leg braces/boots today for the 1st time from Mr. Spencer. I think Dori remembered him from his duet with Chris the last time we were there. We are easing them into wearing these... eventually they'll wear them for about 6-9 hours per day. Today they wore them 2 hours on, 2 hours off, over and over. I told the Ben and Alex it was the babies' Ironman legs. They thought that was cool.

Then at lunch I went to hear Jim and Jill Kelly. Jim Kelly used to be the QB for the Buffalo Bills in the 80's. They wrote a book Chris bought me last week called "Without a Word". Their son, Hunter, was born with a lysosomal storage disorder called Krabbe Lukodystrophy... it's in the same family of genetic terminal illnesses that the babies have. Hunter never was able to utter a single word but his love for his parents, his sisters and life were very evident to all who knew him. Their book and the message they shared today were truly SO inspiring and such a blessing to Chris and I. Chris was gone today working in Nebraska, I really wish he could have heard their program today. So many things in their book is so familiar to us, and it was great to read it and hear it and be moved by it.

When I went to get my book signed, I thanked them for being a strong voice for the parents of children with genetic disorders. I showed them a picture of Dori and Wynnie, and then I lost it. (Tears, lips wouldn't stop quivering, snot running down the nose, the whole nine yards). But they were kind and I was so embarrassed. I started off so composed and by the time they signed my book, I left the line looking like a hot mess, sobbing. N-i-c-e.

On another note, plans are in full swing for the most awesome Halloween party this side of the Mississippi. For those of you that went last year... you know what I'm talkin' about! Party for a great cause! To raise money for the National MPS Society!! And see if this is the year I convince Chris to go in drag!! (ha ha!) Sponsors are getting locked in, prizes are being secured, and secret costume planing are all underway. Saturday October 30th is the big night, so get ready!

August 2010

2010-08-30T11:31:00.000-07:00

Summer flew by in a flash... can't believe Ben and Alex are already in their 2nd week of 1st grade, our swimming pool is finally done (kinda), and the babies will be turning 2 just around the corner! (Oct. 27th)

Ben and Alex are once again enjoying football, Chris is the head coach. The Colts are thus far 2-0, and we have some really fast kids on the team who do a pretty good job of listening so we may have a good chance of going far in the play-offs this year. The big boys are in play-date heaven... now that our pool is in we randomly bring home at least 1-2 kids with us everyday after school to swim. We never keep the home phone outside so if you need to get a hold of us, send over one of those message planes cuz now we basically live outside. Even the babies like hanging out there now.

The babies will be getting new tubes in their ears sometime this fall, we first need to clear it with our cardiologist to make sure they are OK for undergoing surgery. Our cardiologist, Dr. Wright, retired last month :( so we are now moved over to another cardiologist who took over her patient load. We might also get adjustments made to Wynn's hearing aids once he's under for his tubes procedure. Our pulmonologist also needs to chime in about this because since their airways are restricted, we will eventually run into the problem of not being able to intubate them during anesthesia.

Next month the babies will be wearing braces on their legs to help their muscles retain the position they are supposed to be in (not little ballerinas like they like to do now), and to help them gain stability when practicing standing. The next goal after wearing braces for 6-8 hours a day for months is eventually get them into standers (a thing that straps them in by the chest, waist and legs) so they can get used to bearing weight and gaining balance in the standing position. That'll help them in many ways.

First day of School--- 1st Grade!!

Our speech therapist informed me she was going to put in a request for an assisted communication device for the babies. She feels their fine motor skills may not catch up to allow them to do sign language, but she wants to wait til January to see if their work in occupational therapy helps improve those hand movements. If not, she is confident that their cognitive responses have proven that they can learn very well how to use an assisted communication device. She has worked on them for weeks doing "tests" with picture flashcards of our family... she says "where is mommy?" or "where is Ben?" and the babies these past couple of weeks have scored 100% every time she tests them (they'll reach over to the answer in Wynn's case, or stare at the correct flashcard in Dori's case), so she concludes that cognitively they are doing great... well enough to express choices so she says they are ready for this next step. I was so excited when she explained it to me that I started crying! It's a neat little machine with buttons on it that they can press when they want to express something like a bottle, a diaper, a sad face, a toy, etc. Here is a picture of one I found when I googled...

Assisted Communication Device: This one would be for someone older, I don't think the babies would be asking for a cup of Joe, or a boom box. Or maybe they would (?) I guess we've never asked them

Wynn has been having a little hard time breating (he sounds like Darth Vader), so we're taking him into the pulmonologist tomorrow to see what's up. Other than that, we're all enjoying the slightly cooler weather (cooler is mid-lower 90's for us), the fun pool to splash around in, and the new friends the boys are making. Stay tuned, we'll look forward to posting pics once the babies get their leg braces.

Atlantis and Summer Fun

2010-07-29T12:43:00.000-07:00

Summer is 1/2 way over and it's been a fun one for all of us. Ben and Alex are excited for our pool that should be finished up in 2 weeks, the babies have made great strides at pt and speech therapy, Chris is counting down the days for the Huskers to take over their new conference, and I'm happy that our house is finally getting closer to having our projects complete.

Our family gave a good plug to the National MPS Society by a recent full page article they did about "our favorite things". Everyone looked really cute in the picture, including Chris and I. ha ha!!

http://www.dallasnews.com/sharedcontent/dws/fea/lifetravel/stories/DN-lbh_mytown_0726gd.ART.State.Edition1.3cc0e54.html

The babies have done really well at their 6 times/week PT, OT, Auditory and Speech therapy. It's amazing how much more social Dori is now that he has a couple of favorite words. He loves to sing and to be sung to, and he'll cheer for himself often when he does a great job. Wynn is doing well too, we're just trying to get him caught up in the 2-way communication game. They both fought a little bout of pneumonia and ear infections earlier this month but have recovered just fine.

We had a total blast in the Bahamas. I've attached a video here with our pictures and video we took. The boys loved it so much that Ben asked if we could move and live there forever. The babies did so fantastically well on the plane ride and were so relaxed and chill during all our trips to the pool, the markets, and the restaurants to eat. They were never fussy, only when it was time to go to sleep. We were so proud of them, we just might start jet-setting with them more often. It was very helpful to take Aury with us on vacation. She stayed with the babies at nap time while Chris and I ran around Atlantis with the boys and when we went out at night. Hopefully she had fun, too.

The babies continue to do great with all their appointments. Both of them got abdominal sonograms this week since their GI doctor felt something in their abdomens, but he didn't seem overly concerned... he explained that he wants a baseline sonogram to watch how they progress down there. We're still waiting to hear the results from that.

Ben and Alex have done a couple of camps this summer, and still have a couple of more camps to go. Chris is coaching Ben and Alex's football team, I'm getting ready for work this fall and coordinating the PTA "Meet The Teacher Night" next month. Everyone get ready... plans are underway to for the 2nd annual MPS Halloween party co-sponsored by our awesome neighbors. Come on down to visit ... the pool will have a lounge chair and a margarita with your name on it!!

Cardio Update

2010-05-26T20:26:00.000-07:00

Dorian and Wynn had their cardiologist follow-up this morning. The good news is that although the leaks in their heart are getting worse, both babies have relatively good cardiac function. These little guys have managed to keep their hearts working fairly well despite everything. There's no doubt that Dori and Wynnie are our lil heroes.

The doctor bumped up their meds to account for their weight gain since the last time they saw her. Dorian has a moderate mitral valve leak, and a mild aortic valve leak. Wynn has a moderate aortic valve leak as well.

Tomorrow we have a new home-based pediatric therapy company coming out to do an assessment. Although we have very close to our current OT & Auditory Therapy therapists, we have decided to go with a new therapy agency for OT, Speech, Cognitive Development and finally adding PT to the mix. We'll keep our current Auditory Therapist. Wynnie just LOVES her and she does such a wonderful job with him that I don't think he'll let her go anywhere. But want to get more frequency of therapy and also challenge the babies more in getting them to maximize their mobility, and our current agency just has a different philosophy on that than we do.

Our CPS case has been officially closed from our 1st sleeping nurse, so that is nice. Although I caught another nurse totally sound asleep on the babies' bedroom floor - I decided to NOT tell the agency because then we'd have to go through all that stuff again and we don't have the spare time to entertain yet another CPS investigation due to another nurses incompetence. On an unrelated note, that same sleeping nurse (#2) later had her car vandalized in front of our house ( along with 4 other cars on our street) and she hasn't been back (strange since our neighborhood is by no means the "hood"). Aside from me having to walk over her sleeping-self to help Wynn who was crying, she is usually a pretty good nurse so I didn't want to fire her.

We are all getting adjusted to our new au pair, Aury. Ben keeps asking when Anna is coming back and he is giving Aury the same cold shoulder he gave Anna the first 4-5 months she was here. In fact, I was sadly surprised when the boys tore her room up -- lotion all over her mirrors, squirted water all over her floor and bedding, and her cell phone ended up in a puddle of water. What vandals Chris and I have!! ha! They had to apologize and have been grounded this past week for their "crimes". I asked them tonight "Ok guys, you won't get in any more trouble that you're already in, but I want you to honestly tell me why you did those mean things in Miss Aury's room." Ben still swears he had nothing to do with it and that he was only trying to clean up Alex's mess. Alex explained to me he found a water bottle and his brain just kept telling him to squirt squirt squirt. Riiiiiight. Darn brain!! I had to try my best to not crack a smile when he was telling me this.

Teaching Sunday School (CCD) is over for the summer so it was nice to sleep in and not do a darn thing this Sunday with all my boys. Chris is coaching basketball for Ben, Alex and Ben finished up football, and Alex keeps doing well at his Kumon tutoring place. Lots of big milestones this upcoming month. This weekend Chris and I celebrate 12 years of marriage! Then next month Chris turns the big 4-0. I was asking the boys what we should get him for his birthday and they said "Let's get him a lego set." I don't think Chris wants to step on any more legos barefoot in the middle of the night so we'll be thinking of something else.

We are all doing well and enjoying the idea that we'll be in the Bahamas next month with the 4 kiddos enjoying some peaceful family time. Chris was telling the boys that this trip would be Dori & Wynnie's 2nd time in the Bahamas since the last time we went I was 3 months prego with them. Ben and Alex got a kick out of that. We have lots to be thankful for and we'll have plenty of reason to celebrate while we're there. Thank you all for keeping up with us and we hope your families have a great start to your summer as well. Come and visit!

MPS Awareness!

2010-05-11T08:25:00.000-07:00

April 2010

2010-04-23T19:03:00.000-07:00

Spring has flown by with Ben being in 4 sports, Alex being in 1 sport and 2 after school activities, and the babies always busy doing their thing. Our time with Anna is almost over... she leaves in the 1st week of May. Our new au pair is ready and seems to be excited to be joining us in just a few short weeks. It'll be weird not having Anna here anymore... she really has fallen in love with the babies. With that American family that sent back their 8 year old Russian boy they adopted and Russia threatening to freeze all adoptions, Alex was concerned Anna was leaving because of that. Funny. For her going away present, we took her to the Alicia Keys concert, she loved it.

Dorian and Wynn have both made lots of good strides in their mobility and growth this month. They both roll around all over the place on the floor (fast!), both have grown quite a few teeth and both are always curious about the world around them. Our physical therapist today was so happy for Dori. She said that ever since he's been sleeping with the bipap, she has noticed his alertness, happy mood and greater tolerance for therapy has dramatically improved. Since we see him everyday we haven't noticed the huge difference, but it makes sense that a well oxygenated baby is happier and healthier. We are so proud of him.

Since the last time we saw our GI doctor, they both gained a pound in one month! That's a big deal since that means Dori won't need a feeding tube for the time being.

For those of you that keep up with us on a regular basis, you may have heard about some of our nurse issues. We have night nurses coming in to monitor Dori in the evenings since he couldn't tolerate nor sleep with his bipap mask. The nurses are also in charge of monitoring his pulse-oximeter (sp?) to measure his O2 and cardio. If O2 levels drop too much, they are supposed to administer O2 via the tanks we have next to his crib.

The first two nurses they sent were very nice people, but NOT good fits for us. The first one would pace around nervously and say over and over "Oh my God! Oh my God!" when Dori would start crying. The second nurse was sweet as could be but during her shift Chris and I had to wake up 6 different times to help her through the night.

But the third nurse who we had to fire is what this past month's drama has been all about. I found her asleep on our couch with her arms spread wide open as well as her mouth... only in the 2nd hour of her 12 hour shift. I didn't want to embarrass her and wake her up since the babies were safely sleeping in their cribs while she was snoozing, so I left her alone. But when I called the agency the next morning to tell them not to send her back, they asked for a reason and I told them it was because of her sleeping. The storm of crap began after that. I had no idea that a nurse sleeping on the job is considered child endangerment so CPS (yes, Child Protective Services!!!) was called in by the agency to report the "incident". According to the agency, they have to "self report" violations such as sleeping or else they could get their license revoked.

Alex was still at school at his science club, but Ben was home already when the CPS lady came. The CPS agent asked me all the details about the nurse, how she was sleeping, etc. Then she asked Ben directly if she could talk to him. He said "after I'm done eating my snack please." Then once he was done, she started asking him about "good touch" and "bad touch" and if anyone has ever "touched" him in his privates. Ben's eyes were huge as saucers and he kept on looking at me very uncomfortably while he sat on Chris's lap. She asked him if we ever left him alone, if we make him food, and what we do to him when we punish him. All the while, Chris and Anna and I are all staring at each other as if "Is this crazy woman REALLY asking these questions!?!"

But I was SOOO proud of him. He answered everything like a big boy and I was surprised at how surprisingly articulate he was for 6 years old. Then she proceeded to take pictures of all the kids, and note their teachers names, pediatricians name, etc for their "file in the system". Chris and I are outraged. We don't want our precious boys in the damn "CPS system"!! We didn't feel like we could say anything to her in fear of retaliation for what she may perceive as hostility or dishonesty on our part and she was writing down everything we were all saying so I felt like anything interpreted the wrong way could come back and haunt us. The LAST thing we wanted when we fired the nurse was for CPS to launch a full blown investigation AGAINST US when WE didn't do anything wrong! I told the CPS lady I never thought the babies were ever in danger under the nurses care and I didn't want this to snowball into something unnecessarily or for them to call Ben and Alex's teachers since they have nothing to do with the babies or the nurse. I am so embarrassed that poor Ben had to go through that, and we are so so angry.

At this point, all we can do is wait and see what happens. Sadly, the children that truly DO need an advocate to stand up for them against their abusers are rarely ever identified and helped out of their situation, so it's a shame they are wasting their time and energy on our family.

So I don't want to sign off on such a negative note... so I'm just glad Ben didn't tell them I threaten him and Alex often with a knuckle sandwich and tell them that if they don't know their numbers and letters they'll be homeless like people outside of the Mavs games. I supposed that would be considered mental abuse so big sigh of relief for Ben keeping it PC. HA!!!

Cardiology and Respiratory Update Feb. 2010

2010-02-24T06:10:00.001-08:00

The babies had a cardiologist visit last due to our pediatricians concerns on Dorian’s heart murmur sounds getting worse. Chris had to take the babies by himself since I was en route to the airport for a couple of presentations in Rapid City, South Dakota.

After doing a few rounds of tests, Wynn’s results came back well. No need to increase his dosage of Enalipril.

Dorian’s results were concerning to the cardiologist. She agreed with our pediatrician that his heart is indeed leaking at a worse level than it was before, and his left ventricle has enlarged more too. Since his heart is not functioning properly, this may be the culprit of his breathing difficulties (more on that later). First line of action is to add a Diuretic to his regimen. The combination of his heart working too hard and breathing problems make heart failure a high risk for him our pediatrician stressed, so we are moving aggressively with meds to help alleviate the workload on his heart. We’ll try this for three months and see where we go from there. Big prayers for Dorian’s ticker please!

Dorian went through another sleep study last week with a cpap (oxygen mask on his face). He totally HATED it. All night long he either cried or tried to yank the mask off. And I don’t blame him! Between 8:30 PM – 5:30 AM he literally only slept well for maybe 1.5 hours total. We should be hearing back about the results sometime this week. More than likely he’ll need to sleep with this cpap mask to be able to get enough oxygen to his heart, brain, etc.

Both babies are really jabbering away… when I’m working downstairs in the study it’s funny to hear them yap yap yap away with Anna. Oh yeah, Anna and the babies spend all their time upstairs now since we are getting new flooring and a new island in our kitchen. Too much dust and commotion for the babies downstairs so they have moved up to the penthouse level in the room next to Ben and Alex, ☺

Both babies are sprouting their teeth (little nubs of teeth actually) and they enjoy chewing on everything to make their mouth feel better. We still have some issues of both babies bleeding out of their ears so we’ll be seeing the ENT soon to get to the bottom of that. Dorian is loving to move around on his tummy and flipping over, and he is very stealth about it. There is nothing stealth about Wynn… he is content with hogging up as much attention as possible and he’ll let you know it without hesitation. So we send Ben and Alex on a lot of “Go talk to Wynn” missions if we’re tied up with Dori. They are just so darn cute and funny!

Alex started Kumon yesterday to work on his writing and math (Kumon is an afternoon learning center, and our neighbor 8 houses down from us owns the Kumon center less than a mile away from our house). Alex loved it! He said his tutor thought he was in 2nd grade. That made him feel very smart and proud. Ben will take a break from Kumon until summer since he tested two levels higher than average. I’d like to say he gets that from me (ha ha.. jokes!). Both boys started football yesterday on a new team in a new league. It’s still fun to watch Ben at boxing, he is enjoying it once he gets there but he complains about going. We’ve been chanting “USA” a lot at home watching the Olympics with the babies upstairs. Anna is hooked on the Olympics, so I’m glad I have someone to always watch it with. Ben and Alex had a blast at their birthday party a couple of weeks ago. They got lots of neat stuff and most especially enjoyed hanging out with their friends. We’re really proud of our big boys… they always have a ton of questions, always argue with each other like two cranky old men, but always show us and the babies all sorts of love.

Alex’s funniest/sweetest thing he said to me yesterday: I was dressed up to give a speech in downtown Dallas yesterday morning. While they were eating breakfast Ben asked where I was going and I told them “I have to give a very important speech in front of 1,200 people all by myself!” Ben’s eyes opened real big and Alex closed his eyes. He opened his eyes back up and said “Mami, I’m so proud of you that when I closed my eyes I cried a little.” I busted out laughing, it was so funny and sweet!! I guess he gets that from me because when I’m proud of them I start bawling!

Once lost now happily found in search of sleep

2010-02-16T20:00:00.000-08:00

Here I am Celebrating Fat Tuesday at the pediatric sleep institute with Dori while Chris holds down the fort at home with the other boys. This time it's to measure Dori's tolerance for a cpap. He hates it and seems uncomfortable but we gotta give this a try to help him breathe at night.
To pass the time I was researching quotes about family on my iPhone and I found one that I first stumbled upon before Chris and I ever had children. And even though I hadn't experienced maternal love first hand, the first time I saw this quote it teared me up. I was so inspired to find it again I needed to share.
"Before you were conceived I wanted you. Before you were born I loved you. Before you were here an hour I would give my life for you. This is the miracle of life." - Maureen Hawkins

January 2010

2010-01-28T20:46:00.000-08:00

Dentist Time for Wynnie

Dori enjoying dentist time

Dori and Wynn dressed up for New Years to hang with bro.

Hoping everyone's new year is off to a great start. Ours is starting off to be a good year with lots of projects around the house, extracurricular curricular activities with Ben and Alex, work keeping Chris and I really busy, and lots of snuggle time with the babies.

After Christmas Dorian started saying DaDaDa and MaMaMa sometimes and just two days ago Wynn surprised us with BaBaBa (which according to Anna, BaBa means grandma in Russian). We completed yesterday our annual review with our team of therapists (we had 2 Occupational Therapists, 1 speech therapist, 1 auditory therapist, and 1 cognitive development therapist present at the evaluation). It was really neat to compare what our goals were for the babies last year -- Dorian met nearly all his goals from last year except turning/rolling over. But he can now eat/swallow without assistance of tubes or feeding machines, he enjoys his bath time instead of crying like he used to, he reaches for toys, he engages and responds to people when they interact with him, etc. Wynn is the show off... he met all his goals for the year. So we look forward to continuing working with our harem of therapists :) Luckily for us they all come to our house and the boys are sweet on all of them except the Occupational Therapist since they think therapy time with her is torture. She's the nicest gal... she just pushes them to do more with their body than what they naturally do (stretches, loosening up joints, bearing their own body weight on legs and arms) and they just scream until she is done.

Today the babies had their second visit to the dentist. He recommended the babies see an oral surgeon about cutting down their overgrown gums to continue allowing more teeth to sprout out instead of them being in a perpetual discomfort of teething. Wynn has two baby "fangs" on his top, and two little teeth on his bottom (it's his homage to Chris & I's favorite show True Blood), and Dorian's teeth are begging to come out but just haven't broken through the gums. Surgery isn't urgent so he said we can wait a few months to have it done. Three dental assistants gathered around the babies and were lovin' on them during the whole visit and saying how they couldn't believe how calm and laid back the babies were. Proud mama and daddy after those comments!!

Dorian last Friday had to participate in an overnight sleep study at the pulmonologist's sleep institute to measure whether Dorian is getting safe levels of oxygen while he sleeps. I spent the night with him and knew all the alarms going off during the entire night were not a promising sign. Normal oxygen levels for people is usually around 98-100%. Borderline is considered 92%. For Dorian he kept dipping throughout the night to 65%, and that is considered severe sleep apnea which is not uncommon for children with constricted airways or facial abnormalities. Our first intervention will be to possibly have his tonsils removed to help free up space in his airway. Second intervention would be to install a CPAP or BiPAP on him. They are basically a face masks or nostril tubes connected to a machine that delivers pressurized air to his lungs. It doesn't breathe for him, but it helps deliver the proper amount of oxygen to allow his lungs to work right. He would only wear it at sleep/nap time. We have to wait until we see our ENT for him to determine the next step with our pulmonologist. We'll be seeing our ENT as soon as we can get a slot in his schedule early next week. We need to fix this very quickly since O2 levels as low as Dorian's put too much strain on the heart and don't provide enough oxygen to the brain to allow normal brain function and development.

On a lighter note, Ben and Alex are doing great. Ben sat down and read us a book about sharks for the first time this weekend. He persistently sounded out every word until he could figure it out, and we were so proud!!! I almost cried!! He really likes writing and playing games on his computer. He likes math and letters games. When he's doing his letters/numbers he announces to us "I'm doing something very important!". Boxing and basketball are going really well for him too. He needs his own iCalendar and chauffeur to be his taxi to all the stuff he does.

Alex is loving having friends over to play, having dance parties with me (he only likes Michael Jackson music), and lovin on his baby brothers. He started science club this semester and he is really excited about that. At least once a week he gets sad and tells me he'll miss me when he's married. I told him I'd still be around and I'd take care of his kids for him, and even cook for him and his wife every now and then. Tonight he almost started crying at Target about it!! I don't know where that's coming from but Chris tells me I'll probably be a slightly overbearing mother-in-law so I'm sure Alex won't have much time to miss me ... ha ha ha!!

Anna will be going back home to the Ukraine to finish her studies and get back to her normal world in April. We have already selected our new au pair, Aury. She is from Barranquilla, Colombia and will be joining us as soon as Anna leaves. Wynn will REALLY miss Anna. When she feeds Dori he insists that she put him close enough so that he can touch her with his feet. If not, he'll scream and holler until she does. Then once Dori is finished eating, then he is happy being anywhere as long as Dori isn't getting all the attention anymore. We think she's his first crush. :)

2009-12-23T17:24:00.000-08:00

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.

October/November 2009

2009-11-16T08:45:00.000-08:00

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

2009-09-02T09:32:00.000-07:00

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!

2009-06-29T19:58:00.000-07:00

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

2009-06-06T05:03:00.000-07:00

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!

2009-05-29T22:29:00.000-07:00

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

2009-05-06T21:21:00.000-07:00

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!

2009-04-01T13:32:00.000-07:00

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! :-)

Spring: The Gift of Perspective

2009-03-24T13:44:00.000-07:00

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.

2009-02-19T10:44:00.001-08:00

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.

Priceless!

2009-02-01T16:59:00.000-08:00

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.

2009-01-19T20:22:00.000-08:00

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.

2009-01-09T17:40:00.000-08:00

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 www.mpssociety.org/content/4043/ML_II__ML_III.

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!