Prince William got married, Prince Ethan moved out of the PICU!

Yep-that's right, Ethan's out of the PICU and back on 4 Sutherland tonight.  I'm sure Kate and Wills understood us missing the Royal Wedding whilst the bald kid was hospitalized.

I didn't want to put every update on this blog, as Ethan has a carepage dedicated strictly to him-so feel free to follow the events that have led us to where we are today by following this link-it also goes back quite a few years and if you need some help sleeping, feel free to read all about our little Prince!

http://www.carepages.com/carepages/EthanR/updates

if you've never read his carepage before, you will have to create a log-in with password-don't worry no crazy emails or spam-just a great way to keep friends and family apprised of everything when the last thing I want to do is call and tell the same story to 15,000 people. 
Yep, cause I know 15,000 people who would want to know what's going on in my kid's life....

Anyway-missed the Royal Wedding today, so I am planning to throw my own version of one real soon-
what girl doesn't want to wear her wedding dress again?  and what girl doesn't want an excuse to wear one of those crazy-ass hats?  I have the solution.  Calling all commoners, don your wedding dress, flip-flops for comfort and the craziest hat you can find or create and let's have a Royal Reception!   Complete with tea and crumpets and beer, cause what Princess doesn't like a good, cold beer?  Bring your Prince, or not, actually probably not, we will surely have more fun without them anyway! 
So, let me get the Palace cleaned and the little Prince home and I'll set a date,

A Royal date, and you're all invited!

Ethan with his Zhu Zhu pet on his shoulder!

3rd admission in 11 days....I'm feeling 40 now!

Yep, since my last post about Ethan being admitted, he has gone home and come back twice in 11 days.  After the first admission he was home for 2 days and at his follow-up discharge appointment in clinic on Friday the 22nd, they took one look and listen at him and put him back in over the weekend.  That admission they finally did blood work because his chest x-ray again showed no solid pneumonia, and his virus panel was negative for everything.  The blood work showed an elevated white count of 17,000 (high end of normal would be 11,000) which indicates an infection of some type.  They started him on some antibiotic and sent him home on Sunday once they got his oxygen weaned down to room air while awake and 1/4 liter while sleeping. 

Sunday day and evening he was marvelous!  Back to his normal self, laughing, rolling around, just all around acting like a goof ball.  I put him in bed and on 1/4 liter of O's just to be safe, only to be woken at 1am hearing him fuss.  When I checked on him, he was working really hard to breathe, looked to be a little puffy around his face and neck, hands and feet, and began retching.  I turned his oxygen up to 1 liter and he seemed to get a little more comfortable, but he was very fitful the rest of the night and pretty miserable.  All day Monday he was not terrible-but not great.  He continued to retch, so I kept turning his feeds down throughout the day-but when the vomiting began at 5:30pm-I knew we weren't going to be staying home for much longer.  At the ER his sats were 82 on 1 liter so in he came.  He is on 4 Sutherland room 3 right now being followed by the Purple Team again whose attending is Dr. Keith Mann.  I was thrilled to find out that Dr. Mann is the attending this week and going to be taking care of Ethan!  He is the physician who began the Family as Faculty program where residents are assigned a medically complex kid to home visit, follow to clinic visits and just try to get an overall view of what it's like to have a kiddo like Ethan.  We had two residents assigned to Ethan whose rotation was in February where they visited our home and got to know a little about our special guy and our lives.  Anyway-I am a HUGE supporter of the program and thus of Dr Mann and feel lucky he's taking care of us this week!

Where are we right now???  Ethan's blood pressure is running a low, his heart rate is high, his oxygen is on 1 liter to keep him above 90, he coughed most of the morning so far, and his hands, face and feet are puffy.  His belly and chest are distended now too.  His white count is up to 27,000 after having been on antibiotic for 4 days indicating it's not touching whatever infecction he has.  His BNP, or brain natriuretic peptide, one of the labs they did last night was elevated-WAY elevated-3,350, whereas <100 is normal.  This is the lab work used to indicate that congestive heart failure is likely.  Soooo, he is getting an echo today, an x-ray of his belly, some more lab work, and a dose of lasix.   He has never been in congestive heart failure before, and it just may be some right sided failure this time due to his respiratory compromise, or it may not be.  The echo will give us more definitive answers, and what we see him do with the lasix.  Right now we are in a holding pattern.  I will update as we know more.  In the meantime we are hanging out listening to Taylor Swift and I'm catching up on the first 3 seasons of Grey's Anatomy!

Below are a couple of pictures from my Fabulous 40th Birthday party from last weekend-I have tons more-and will load them on my FB page soon.  I want to say Thanks to all of my family who helped pull it off while Ethan was in the hospital!  My Aunts Kathy and Mici, who stayed with us last weekend, helped get all the meat smoked and made the beans and salsa and corn confetti salad, did laundry, and dishes, and kept Parker busy while we ran around.  My cousins Tony and Jim and sister Kim who set up the clubhouse tables and decorations and made the place look fabulous!  Michelle, who made all the delicious cupcakes!  My Mom, who made the potato and macaroni salad, and cut and blinged cupcake toppers.  My Dad and Teresa who went down to the hospital and sat with stinky during the party so he wouldn't be alone.  Thank you, thank you, thank you everyone!  It was a wonderfully, fantastic party that won't be soon forgotten, I love you all very much!  To all my friends and family who atttended-thank you for the gifts and cards and for being there.  One thing I've learned in the last 40 years is that I have some pretty amazing people in my life that I wouldn't trade for the world!! 

Gotta run-Ethan is not comfortable and is wanting to be held-which is quite an ordeal in itself!



Day 3-whiny + pitiful = exhausting!

love the tongue!

Sunday was a long day, especially since we didn't get to bed until after 3am because Ethan could not get comfortable and situated.  He was pretty sad-if we held him, he wanted in bed.  Once we put him in bed, he wanted to be held....and so on and so forth.  Exhausting is the easiest way to describe it.  For us and for him-since he's the one who doesn't feel good, I'm sure it's way worse for him!

Brian, Parker, my Mom and I went to Hailey's last volleyball tourney of the club season, while Grandpa Chuck hung out with E for a while.  Hailey's team took 3rd place and got bronze medals-and we got lots of great pics-will post soon! 

The Purple Team decided to go ahead and do an IVIG treatment on stinky, and started the Gamunex at 10:30 this morning-it just finished, and they will have one more set of vitals to take on him in 30 minutes -then he can be left alone to sleep.  Since he has never had an IVIG treatment, it had to run over 12 hours with vitals taken every hour-whereas normally it would only take 6 hours, or an hour if we do it subcutaneous at home.  I don't care how long it takes-as long as he's getting it and showing no signs of an adverse reaction-he can continue it at home! 

His oxygen has been weaned down to 1 liter-and he's maintaining in the low 90's.  He did have a bad episode this morning where he would only mouth breathe, and they had to put a mask on him and crank him up to 3 1/2 liters for a few hours.  He seems to be over that little meltdown now thank goodness-but that doesn't mean he spared anyone any other temper tantrums-those seem to be the norm today.  I'm hoping he wakes up Monday morning in a much better mood.  The only consolation for him right now is for us to ignore him and crank up his Taylor Swift CD-did I mention how much he LOVES Taylor Swift.  Turn on her new CD-thanks Phillips Family for getting it for him for his b-day-and he stops wimpering for a while, or at least long enough to listen to his girlfriend sing for a while! 

So that's where we are hanging for now-still no virus panel results back, and the adrenal insufficiency hasn't been determined yet to be worse or not-we should find all of that out tomorrow.  He's not out of the woods yet, and I will update as we know more.  In the meantime-Ethan sleeping means Mom needs to sleep, and he's sawing logs right now-so that 's my cue....

Please keep Brian's long time childhood friend Steve in your prayers, his Mother Marty (and like Brian's second Mother) is fighting Ovarian cancer right now, and things are looking pretty grim.  They live in Tennessee, and as much as Brian would like to go down there and show his support, we kind of have a full plate here right now.  Steve's dad, Roger, was my and Brian's wedding pastor and they have a very special place in our hearts, so please keep them in your thoughts and prayers for peace and comfort in the coming days. 

love, hugs and prayers,
K

Someone told Ethan we have plans.....



Ethan riding the big rig to the Mercy!



It's been a long time since we've had to keep our upcoming plans a secret from Ethan, but we seem to have fallen back into our old familiar ways of needing to do just that.   It started quite a few years ago....

we have plans to get family pictures taken?  Ethan gets sick and gets admitted.

we have plans to go to the Weston Irish Fest?  Ethan gets sick and gets admitted.

we have family in town for the holidays?  Ethan gets sick and gets admitted.

we set the date for Ethan's birthday party?  Ethan gets sick and gets admitted.

Seeing the pattern here??? We used to joke around that we couldn't share our plans with Ethan, or discuss them out loud around him-or we would inevitably be jinxed.
Well, leave it to the boy who loves his routine and adapts terribly to change-I have plans to run, and I mean RUN the Trolley Run for the first time ever tomorrow morning (I've walked it pushing baldy in the stroller for years)AND guess who's sick and in the hospital? 
My FAB 40th B-day party is next Friday, and we have some of my favorite relatives coming in town to celebrate with us, and guess who's sick and in the hospital?

Yep, you guessed it-Ethan. 

I know what jinxed us-we were in clinic on Tuesday doing the boy's intake interview for the Medical Coordination Clinic-and he was a PEACH!  I mean charming as heck!  Smiling, laughing, showing off for the new staff, following commands when we would ask to see his 'piggies' or tell him if he wanted out of his wheelchair he needed to raise his arms.  And he was ON!  We bragged about everything Ethan-height, weight, feeds, pooping, peeing -you know, all the important stuff.  I *gasp* even mentioned that I would like to see about getting back into the amazing Jodi Gamis -OT Feeding Specialist Extraordinaire-to work on some oral stuff that would maybe eventually lead us into some oral intake again someday.
 That was Tuesday morning. 

Wednesday was good. 

Thursday bad. 

Friday TERRIBLE.  He came home from school on Thursday with a note that said he took a nap that morning and another one in the afternoon which he had to be woken from to participate in music.   That should have been our first sign...which we ignored.  First mistake. 
The second one was when Brian put him to bed at 7:30pm that night and he was still asleep when I got home Friday morning at 7:30am and we didn't take him in then.
Needless to say, I was not surprised when Brian woke me at 12:30 and said we've got to go NOW.

Rolling into Children's Urgent Care North with a kid like Ethan usually gets you taken right back.  Rolling in there with a kid like Ethan whose sats are in the 70's gets you downtown in an ambulance pretty darn quick! 

So, here we've been since Friday afternoon.  On 3-5 liters of oxygen at any given time, lethargic and crabby. 
Negative for Flu, RSV and Pneumonia-just some 'peri-hilar thickening and haziness' on his Chest x-ray.  Pretty normal for the bald kid.  Sats in the 70's-not so normal-even with 'hazy' looking lungs. 
He was initially admitted under Dr. Kaine-Ethan's Cardiologist for fear he was in congestive heart failure.  BUT-upon admission Dr Kaine came in and took a good look at E, and felt that CHF is not the case right now(Thank goodness) and transferred him to the General Peds team with the agreement that if anything changed over the weekend, Cardio would happily take him back.  We are ok with the transfer-though we love Cardio-I like the Gen Peds team too, and his issues right now are not cardiac related.  What are they related to?  My best guess?  The fact that he has been taken off his Immunoglobulin infusions since January due to an adverse reaction to the concentration of the brand we had to switch him to per our insurance. 
For those of you remember fall of 2007 and most of 2008-Ethan had 10 admissions in 9 months, was a DNR at one point and we thought we would never be bringing him home, let alone celebrating another birthday with him!  We visited Dr Portnoy in Allergy/Immunology and he found the cause....Ethan has CVID or Common Variable Immune Deficiency.  So almost two years later of us doing home subcutaneous infusions weekly of immunoglobulin-we went from all those admissions in 9 months to 2 in two years!  So, do I think that was the fix?  YES-especially since we had to stop the infusions in January and we've had more sick days than normal since then.
Why did we have to stop it you ask?  He started out on Vivaglobin-a concentration of 16%, which he did fabulously with for a year and a half.  They stopped making the Vivaglobin-so insurance switched him to Hizentra-a concentration of 20%.  Less volume, less time involved, but lots more reactions.  Site reactions lasting two days, flushing face antihistamine reaction lasting 24-36 hours, and overall just plain crabbiness lasting from start of infusion until about one day before we were due to give his next one.  All those combined made for a cranky kid and unhappy parents.  Per Allergy-STOP immediately before the reactions get any worse, and wait til you hear from us about starting him on a new one-once the insurance company agrees to that -and to pay the $5000 a month it costs.  And when did we get the phone call that all of this had been approved and he is due to get his first infusion of Gamunex C in the next two weeks???  Monday-yep, 4 days before admission!  Oh well, that's how our boy rolls.
Over the two years on the infusions, he built up a nice immune system, but having been off for a few months now-our luck ran out, and so did his immunity to itty bitty viruses like rhinovirus (the common cold)  and his body's ability to fight them off without needing to be hospitalized. 
The Gen Peds team has contacted Immunology and they recommended checking his IgG, IgA, and IgE levels while he is here, and if low do an emergent IV infusion of IG if needed to boost him up.  Easier said than done if we could actually get a good IV on the bald kid.  Only 4 sticks this time to finally get one in his foot made Ethan a very unhappy boy!   I don't think it will last to do an infusion through tomorrow if needed, but we will hope and pray. 
The Gen Peds team also contacted Endocrine about E's adrenal insufficiency, and the possible need to do some stress dosing of hydrocortisone.  YES was the answer-right away, right now and a higher dose than what we normally do at home during illnesses.  ALSO-they recommend a re-test of his adrenal function to make sure it's not any worse than before and that we don't need to be medicating him all the time instead of just in times of stress-illness, prior to surgery, etc.  Those results will be back in the morning, and the plan may change then.  All of the symptoms Ethan had which brought us in this time coorelate with Addison's Syndrome, and adrenal crisis.  I never put them together, because he has been so well in the last two years.  But when he was initially diagnosed with adrenal insufficiency, we were warned about a possible lethargic state, accompanied by low blood pressure and GI issues which could put him in a coma-like state and possibly death to which we would have to give him a shot of dexamethasone and call 911 immediately.  Fortunately this time wasn't like that -but well could have been had we let him linger on with this illness any longer before taking him in.  Honestly-we are pretty on top of the little guy and he crashed hard and fast this time-I had no idea I would see sats of 70 when they hooked him up at UC.  So I've learned my lesson this time, and I will be the crazy, over-bearing Mom now for a while again, until I feel like I can breathe a little easier.

Sorry so long, lots going on with the little guy-will update as soon as we know more.

Good Luck Hailey!

Hailey's last volleyball tournament of the year is tomorrow-so GOOD LUCK CLUB NORTH 14-5's!  Play hard and play  to win-but most of all, enjoy the fabulous club season you all had!  I am so proud of Hailey's success and drive to get over her injury and back on the court!  Love you Hay Jay!!

love, hugs, and prayers,
K



Spurs & S'mores for our Seven year old!

Ethan Charles age 7 on 3/30/2004 
our very special lil' cowpoke!

Ethan is so done with his party!

love the look Ethan is giving Parker?!?!?

We celebrated Ethan's 7th Birthday in true cowboy style last weekend complete with cowboy hats, bandanas, sherriff's badges and s'mores.  Brian nixed the ponies-so we had to make due with a bandana on the dog!

I want to thank everyone who came and celebrated with us.  It means so much to Brian and I-mostly me-to plan and execute a party for our little guy.  I will admit, I tend to go overboard a little with him-we just feel so lucky each birthday we get to cherish the year we just spent with him, and plan for the next.  We learned a long time ago to never take our time with him for granted, and make the moments count.  So, really, these parties are more for me, as he could care less what everyone is doing at the house-though he does really enjoy the undivided attention he gets!

 Everyone always asks what to get him, and we struggle more and more each year with what to tell people.  So much so that Brian and I were arguing in ToysRUs ourselves -I wanted to get him a play gym with bright colors and toys above him to entice him to grasp at something.  Brian wanted to get him a "toy made for a 7yr old-not a baby" as he wanders down the Power Wheels aisle....sure Brian-let's get him a battery operated jeep, or monster truck-or even a Harley.  We could duct tape him in it and put his foot on the gas and let him go hog wild around the neighborhood.....I would put a helmet on him too-just for safety's sake....really??  WTF are you thinking?  I KNOW he is chronologically 7 years old, but he's not REALLY 7 years old.  Guess who won???  I'll just say Ethan is now the proud owner of a Baby Einstein Ocean Wonders Play Gym-which he enjoys quite well I must say!   Don't get me wrong, these are the exact times that I am sad for Ethan's challenges-buying 'age appropriate' birthday gifts, watching nieces and nephews and kid's friends get ready for baseball season,  having him be the first of 5 kids in our family to turn 7 this year-but will always be the 'baby'.  My nephew Kellen was having a hard time with that himself as he was looking at Ethan's cake with a '7' on it.  He couldn't believe Ethan was older than him and was very confused-Kellen's 7th birthday is in July. 
Anyway, I always get a little melancholy when his birthday rolls around, though I love our Holland-I know I will always wonder what Italy would have been like.....my special needs Moms will get my reference ;)
So Thank You to my family and friends who indulge me every year with crazy themes and birthday cake fiascos, and thank you for loving our litte guy!

I gotta throw out some Happy Birthdays to the other four 7 year olds this year:
My nephew Adam-April 5th
My niece Abby-April 15th
My niece Hana-June 29th
My nephew Kellen-July 17th

Can't wait to celebrate with you all too!