Dec 28, 2014

2014 ... It's a Wrap!


Dan and Beth celebrated their 20th wedding anniversary on December 25. 


 age: 16. 
grade: 10th
height: 5’11”
weight: 190 pounds
hobbies:  reading books (especially military and history), discussing politics and current events and dancing.



age: 15
grade: 9
th height: 5’2”
weight: 85pounds
hobbies: reborns (a collectible type of doll), cake decorating and taking care of Manny.



age: 13
grade: 7
th height: 5’11”
weight: 130 pounds
hobbies: electronics, reading, handy-man things.



age: 11
grade: 6
th height: 5'1 1/2"
weight: 104
hobbies: legos (and Halo), reading and baking sweets




age: 9
grade: 4
th height: 4' 5 1/2"
weight: 60
hobbies: listening to music, exercise, Just Dance





age: 5
grade: Kindergarten
height:  46 inches
weight:  55 pounds
hobbies: ipad (minecraft, angry birds/bad piggies, Catapult King), running in Big Red (his wheelchair), and playing with his siblings


Thanks to all our friends and family for your love, prayers and support. 



Nov 18, 2014

Mystery Solved?!?

When we adopted Manny, we knew we had a mystery diagnosis on our hands. Two years later, we found a part of the clue. But it wasn’t the whole picture.

So 4 ½ years of mystery.  And I think I figured it out this weekend.

Background: Every time food goes into Manny’s digestive tract, he does a weird constellation of symptoms.  They got progressively worse until the point where we had to stop all feeds.  Nothing went in his mouth, stomach or digestive system.  ALL went into his veins (TPN).  That was 3 years,1 month ago.  TPN is not without its dangers, however. We have tried feeding trials along the 3 years but they all resulted in the same horrible symptoms that initially landed us on TPN. 

What were his symptoms: The symptoms were numerous but include having a very high heart rate, a high respiration rate, lungs sounding “wet”, intractable retching, copious oral secretions, a g tube output greater than his j tube input, multiple large diarrhea episodes, etc.  What would ultimately cause us to stop feeding him was the inability to breathe and a heart rate of about 280.  There became a pattern that was progressively getting worse.  I would say to anyone who would listen, “He’s not drowning on his formula, he’s drowning in his own secretions!” Everyone would tell me this was not possible.  But I was watching it happen.

Getting ready for this feeding trial: In January we sought out a specialist. In February we were inpatient at a different hospital and had specialized testing done.  Along the way, we tried various medicines and tried numerous things.  We thought we were ready for the trial. So we were admitted.

Why now: We feel like his proverbial ducks are in a row.  He’s been healthy for a while. He’s been on bipap and is a lot stronger than he used to be. If not now, then when?

The feeding trial: We started fairly aggressively compared to the past trials with rate but did quite short duration.  (By going faster, we can give him a longer break in between feeds.) We started a new medicine that was recommended based on the testing that had been done.  We had a second medicine added to the TPN. 

The happy accident: Tuesday Manny had his old tube traded for a new one. (The old one was broken.) While there, he must have been exposed to something he is allergic to (likely CHG). By Wednesday we started the feeding trial.  It was not going super well.  All the old symptoms were starting.  He was retching, draining from his g port, heavily salivating, sounding generally “wet”, etc.  And then he broke with horribly itchy rash. IV benedryl was ordered.  I noticed that within about 10 minutes of the benedryl being started, ALL his symptoms stopped.  Like a water faucet that had turned off.  6 hours later (presumably when the benedryl wore off) the symptoms returned.  Repeat. Same results. 

The research: I was intrigued. I started asking if Benedryl dried up secretions like that. No was the general thought. But I persisted in researching.  The feeding trial was continued, and by Friday I was convinced we would eventually have to discontinue the trial as he was starting to do all his “old tricks”.  Saturday he got his regularly scheduled iron. They give him benedryl before it.  And what do you know? His symptoms ALL disappeared again.  Like a light switch.  Not this was no longer a fluke, it was a pattern. And I was paying attention.  The first major HINT in 4 ½ years!  I stayed up researching and found a possible diagnosis that fit.  It was an almost perfect match. 

The diagnosis:  As of right now, we have a “presumed diagnosis” of Atypical Chronic AngioEdema.  It’s more common presentation is emergent (quick acting) periodic (like once a year or once a month or once) very large swelling of a body part such as the lips, eyes, etc.  If a person has this affect their airway, they would get Epinephrine. But if not, they would likely give IV Benedryl.   

  • Angio means vessels, meaning related to the blood vessels or lymph vessels, etc. 
  • Edema means a swelling of organs, skin or other part caused by buildup of fluid in the tissues.

In this type of edema, it’s called “Non pitting edema”.  In typical edema, you can push on the affected body part and it will leave an indentation.  This kind does not.  Instead, you’ll just be “puffy”.  (Anyone who knows or has seen Manny will likely know he feels super puffy ALL over.)

Most angioedema is episodic (meaning rarely or hap hazardly) vs chronic (as is Manny’s case).  But in his case, his has been chronic/constant.  And it’s certainly gotten more profound and pronounced over the years. Which means it’s progressive.  (He does have a lot of “exposure” allergies … meaning they get worse the more he’s exposed to them.)

Now how it affects Manny:  Abdomen can be affected in certain situations.  And this seems to be the case for him.  When something travels through his digestive system, it triggers this histamine/autoimmune type of response and his body goes into hyperdrive overproducing secretions.  In his case, it’s also periodically caused swelling of his airway.  We’ve found this out the hard way.  And he is even difficult to intubate these days. 

The “treatment”: He gets IV Zantac for digestive symptoms already.  Which is part of the treatment for chronic angioedema.  He is getting augmentin for his motility.  This can also be used.  And they added Zyrtec.  It’s similar to Benedryl without knocking him out.  Verdict is still out if it will work as well as benedryl.  (So far, it's not as effective.)

This makes his symptoms lessen (not gone).  His gtube output is still too high (basically about the same amount that goes In his Jtube comes out in secretions from his gtube.  He is still retching but not severe.  His heart rate elevates with the feeds but not super high.  His diarrhea is bad but doable.  So in other words, he still has all the symptoms but they are at a “tolerable” level at this point. 

Because he is on 6 hours of feeds that equals 210 ml of fluid, tonight (Tuesday), he will officially drop down on his hours on TPN.  He was on 21 hours a day and we are going to 18.  The first REDUCTION of time on TPN in his life! Those hours he’s off TPN is when he’s on feeds so that his blood sugar doesn’t plummet. 

Discharge:  If all continues to go well with this plan, we could go home Thursday.  Many stars have to align to pull this off.  Medicines.  Machines.  Etc.  But still.  This is crazy!

For the first time in over 3 years, we could be going home ON FOOD!

Future: We will develop a plan as we go.  We will see how well he tolerates this and go from there.  We could adjust the rate up or down, the amount of time he’s feed up or down, adjust the medicines, try more testing to see exactly what “triggers” the angioedema, etc.  We have a lot of options as we go forward. 

Theory: I also have a theory of the cause of all this.  I’ll save that for another time.  But if I’m right, it has implications for many other kids. 

Do we have this 4 plus year mystery solved?!?

Sep 28, 2014

Dear Minecraft Creators

Dear Minecraft Creators,

There are articles all the time talking about how electronics and games are ruining our kids.  These people share how our kids are slower, dumber, less articulate and less social than their counterparts who abstain from electronics.  It seems to be “the” thing to do.  And electronics are evil. 

But I have a completely different take.  I believe electronics make for level playing field.  My kid is better off because of these games.  They give my kid the chance to just “be a kid” without the normal limitations he typically faces. 

Minecraft is a prime example.

When the developers of Minecraft were designing and programming this game, I have a feeling they had no clue what they’d created.  I have a feeling they didn’t know it would be THE hit game of millions of kids, tweens and teens across the country. 

But here’s one thing even they probably still don’t know.  They have made a HUGE difference in the life of my son, Manny and many like Manny. 

Manny is 5. Manny is severely disabled.  He’s in a wheelchair full time.  He can barely use his arms.  (For example, he cannot raise his arms to scratch his nose.) He cannot sit independently.  But he can use his hands if his elbows are propped.  And his mind is quite sharp. 

The only world he can truly interact with are things that are brought TO him.  We can hand him a puppy.  But often the dog is too heavy.  We can bring him a football but he can’t throw it.  We can hand him a tower of blocks but he cannot build with them.  He can go to the amusement parks and watch roller coasters but he cannot ride them. 

But an electronic puppy?  There’s a game where he can pet his dog, play “fetch the stick”, feed him and even clean and throw away his poop.  His dog’s name is Diego and Manny has kept him quite happy for months via the medium of an ipad.  His favorite part is scooping up Diego’s poop, putting it in a bag and throwing it away.

Manny also learned to play football.  He can line up the offense, the defense, the special teams.  He can actually play.  He’s as tall as the other players.  He’s as strong and fast as the other players.  He’s just “one of the guys.”

But Manny mostly loves Minecraft.  In this world, my five year old can just be a kid.  He can build a swimming pool and a castle.  He can build rollercoasters and then ride them.  He can wield a sword and defend his treasures.  He captures sheep in fences he’s built.  He built restrooms with toilets.  Periodically he will say, “Gotta go to the bathroom.” And then he runs over there to use it before resuming play.  (This is a kid who has never used the restroom in real life.)  

Manny loves playing multi-player best of all.  Manny plays with everyone who will play with him.  The more players the better.

In Minecraft, he’s the same height and ability as everyone else.  There’s a level playing field.  He’s just as strong as everyone else.  He’s just as fast.  He’s just as capable.  He’s even as tough as his 16 year old brother and 18 year old cousin. 

On the survival level, he loves to lure people over to him with his sweet voice, “Come here, guys.”  And when they do, he whacks them up-side the head with his sword.  Without Minecraft, I’d have no clue I have an “evil genius” in the making. 

Thanks Creators of Minecraft for the gift!  The gift of being “one of the kids”.  You have no clue how important that is. 

Forever grateful,

Thankful Mom

Manny wearing his Captain America Minecraft shirt.