SPOOKY

The above photo represents a very scary and discouraging issue.   For the past 2 plus years, DHMC has been without a pediatric orthopedic surgeon.  Ambrose had been seeing the previous Dr annually, keeping tabs on his scoleosis and his multiple skeletal issues.  For the past couple of years I have been increasingly concerned that his curve seems to be getting worse.  None of his various specialists paid much attention.  However, recently, after ruling out other culprits, his pulmonologist  felt that his scoleosis may be the cause of his worsening respiratory issues.  When I heard they had finally found a new Ortho Dr., I was very excited for him to finally be seen. I was hopeful that he could perhaps try some kind of brace, but concerned that might restrict his lungs further.  
Well, I was very upset to learn that Ambrose's scoleosis is now measured at 90 degrees and considered severe and beyond repair.  A brace would not be an option for him because of his respiratory compromise and also he is beyond what the brace could do.  I already knew that I did not want to persue spinal surgery as I had been told by a PICU attending that he would never recommend it as he had seen kids like Ambrose do very poorly after such surgeries.  I also learned that it is highly unusual for someone as young and small as he is to have developed such severe scoleosis and that the hardware they use in these surgeries is actually to big for him.  So I was told "surgery isn't even an option."
His right lung (seen on the left above as image is reversed) is very squished.  His ribcage is twisted and sticks out in the back on his right side.  I asked the Dr to look back in the computer to compare it to an older image.  She found a chest xray from 3 years ago and measured the curve at 40 degrees.  So in the 3 years that no Dr was watching, his curve went from 40 to 90 degrees and is now "beyond repair."  It cannot be allowed to get any worse!!!  The only thing that this Dr. suggested was having a custom molded seat back made to prevent him from   curving any more than he already does when he is up against gravity.  We are looking into this now.   I am optimistic.

OK so I cheated a bit on Ambrose' Halloween costume.  I made this gnome costume a couple of years ago for him and he looked so darn cute in it.  Since his new diagnosis last year, following his whole exome study, I have wanted to modify this costume and make him : a  HUMAN GENOME
(G-gnome)

Of course he doesn't have a devilish bone in his body, but I couldn't resist.  He looks so darn cute!

JOY!

When I look into these eyes, my heart is filled with joy.  These are Ambrose's "big eyes".  Often we only see them for short periods of time, but this weekend we have seen them for hours at a time. 

Ambrose is often awake with his eyes closed or rolled back for long stretches of time.  When he has his "big eyes on" he is much more attentive to the world around him.  

                                                   He has been engaging and smiling alot.

This past weekend, we were special guests at a Make a Wish fundraising dinner.  I was asked to speak about what Make a Wish has meant to us.  I am too shy to speak in front of a room full of people.  My husband spoke and did a great job.  I agonized about my decision to bring Ambrose.  I know it would be meaningful for people to see this "wish kid", but I worry so much about exposing him to germs.
It turned out to be a great night which Ambrose really enjoyed.

They had asked if it was OK having Ambrose sit at the front table.  They wondered if it would be too loud for him.  I thought it would be fine since he is hearing impaired.  It turned out to be perfect.  He loved listening to the music and especially the Karaoke.  They had strings of lights up that he could see.

Our friend Winnie came.  She loves Ambrose and Ambrose loves her too.  Our boy had those big eyes on the whole night.  We were there almost 3 hours.  I can't recall the last time I saw him engaged with something for so long.  Words really can't express how much it fills my heart with joy to see him like this.

Ambrose had a great summer.  He had many adventures.  Grandma and Grandpa visited.  He went camping (luxury camping).  We stayed in the Director's cabin at Green Mountain Camp.  He went to the Wellness in Windham festival where he got his face painted.  

He made several paintings which were were auctioned off to raise money for his father's Mission trip to Kenya this fall.  He went for many walks, lost more teeth and got new ones, and had several doctor's appointments, of course.  

We have tried a variety of different respiratory medications.  We are currently doing chest PT am and pm followed by Advair.  He does seem to be having fewer episodes of desaturations and is using a bit less O2 overall.  We are also trialing him off of Synthroid.  

Ambrose is the light of our lives and has been his usual wonderful, snuggly, loving self and we are so grateful for every moment with him!

Kindergarten

Dear sweet Ambrose is now a kindergartener!  He is technically a part of the Academy School, but is home schooled.  We have instituted a new schedule of developmental activities to work into his day.  He also has a daily "report card" (sticker chart) and receives many rewards.  He has an A+!  He has just been wonderful as he always is.  He puts up with it all and participates (sometimes passively).  It is very exciting for him to have reached this milestone!

Well Darling Ambrose is having a good Spring, yet keeping us on our toes as usual.  He has been having frequent sudden desats following position changes, that require bagging and xopenex (an inhaled bronchodilator like albuterol.)   Fortunately, they resolve quickly.   The sudden onset and postional nature makes them appear like obstruction (Ambrose has a history of this which resolved once the correct angled custom trach was created) but the fact that it is resolved by Xopenex sounds like bronchospasm.  To add another layer ontop of it, we have had the windows open and taken him outside alot during this period, which happens to be peak allergy season.  Could it be allergy related?

We are now trying a twice daily inhaled steroid called Flovent.  It will take a while before we see if it helps.  I have also been trying to keep the windows closed and keep Ambrose inside.  We have installed the AC since it has been muggy.

 Ambrose and the children of the Dummerston Congregational Church with the tree they planted.

Ambrose sound asleep, playing his switch activated bongos.  Sorry the video doesn't seem to work.

Ambrose's nurse helped him create this reversible masterpiece with his fingers and feet for Mother's Day.

Another video that doesn't want to play, but won't let me delete it.  It shows Ambrose in is stander playing other LOUD switch activated instruments and he is totally unimpressed.

Ambrose also lost his first molar.  It was not the loose one I've been watching for months, but a total surprise.  I saw it on the back of his tongue after having brushed his bottom teeth and flicked it out of his mouth before he was able to swallow it.  Yikes!  He has now lost 12 teeth.  Only 2 are unaccounted for and most have grown back.  Unfortunately the sharp spikey ones that cut his tongue came back the same way.  In fact, his tongue is crooked, with a deep groove in one side from these teeth carving into it.  A couple of years ago we had a dentist file the two front bottom teeth.  It was quite a scary ordeal for him and he wound up getting his lip cut as he was trying to get away from the drill.  It did stop the daily assault on his little tongue though.  Sometimes it's not easy being Brosie.  But the continuous hugs, kisses, and cuddles help.

Well, we have been home from the hospital for two weeks now and Ambrose is finally coming down to the upper end of his normal oxygen range and is not needing quite so much suctioning.   It really takes him a long time to recover when he is sick.   They sent him home on Augmentin antibiotics, which of course gave him terrible diarrhea and diaper rash, despite using Culturelle for Kids probiotics.
(His White Blood Cell count was elevated at 18, indicating infection.  His cultures grew normal flora, but they wanted to treat because of the WBC count.)  He has been feeling much more like his wonderful self.
I have been telling myself that he needs a hair cut for a while now.  Yet I keep postponing doing it.  It is hard to do as he keeps moving his head around.  I want to get it done with quickly.  His hair is also quite tricky as it is straight in front and very curly/frizzy in back.  I always think, "What have I done?" mid haircut.  It always looks crooked (because it probably is) and too short.  His hair gets wild when it is long and has a lot of personality, which I love.  When I cut it he loses some of that and it emphasizes how small his head is.  I don't know why it always seems to take me by surprise and makes me a little sad.  When his hair is long, it sticks up and I think of him as being like an animal in nature trying to make itself look bigger than it is.    Before and after shots below.

Our 2 year no hospitalization streak has come to an end sadly.

We had a good run.  Ambrose is in the PICU.  He had been sick at home for almost 2 weeks.  The only exposure he had was at hospital appointments a few days prior to getting sick.  I try to be so careful.  It is so frustrating!
Two weeks ago Ambrose began needing more O2, having copious secretions, and having fevers.  Of course it was over the weekend.  By Monday, his fevers were gone so I was hoping he was on the mend.  He has weathered several respiratory illnesses at home the past 3 years, since we have had the ventilator and can give hime CPAP and pressure support.   He always takes a long time to get over something.  Progress is slow, but you see a few good signs and want to believe he is getting better.  I agonize over the decision to get a physician involved, especially since I believe that is how we got sick in the first place.
The other night when I got home from work (at midnight) Ambrose's nurse told me that he was desating while sitting upright and only tolerating laying down in his crib, despite xoponex and some bag breaths.  This was quite concerning.  I put him on his vent for the night and tossed and turned (as did Ambrose) knowing I would have to make a decision in the morning.  When morning finally came, Ambrose immediately desatted upon coming off the vent, despite the fact that he was lying in his crib.  I called his pulmonologist, who was at a conference.  Fortunately her secretary called her on her cell phone, she called me, then the PICU and made the arrangements.  I called 911, for only the 2nd time.  I usually try to transport him myself, but knew they could transport him lying down.  Rescue, Inc. did a great job with him and have just been wonderful to us!  He went to MY hospital, BMH, awaiting transport to DHMC.  Everyone there was great too!  After the obligatory few hr wait for transport, the DHART team came to transport him.  When moving him from the ER stretcher to the ambulance stretcher, Ambrose had a big desat and distress.  After instilling some saline in his trach he coughed up a huge, thick green mucous plug.

The PICU attending spent about 45 min with ultrasound looking for a good vein and using it for guidance in putting in a peripheral IV.  Poor baby had to have his arm taped to a board.  Needless to say, he did not like this.   I do appreciate the care that was taken to try to prevent repeated unsuccessful pokes.  
Ambrose had various cultures taken and was started on IV Zosyn and inhaled Tobramycin.  He had many seizures that day as he had missed some of his seizure meds during his hectic day.  It took 6 hours after he was admitted before they finally got meds for him.  That is my biggest pet peeve.  It should not take that long and that has always been my experience there.
Anyway, my darling did well, grew essentially normal flora and was able to come home Sunday afternoon (2 days later).  It seems that the plug was the source of much of his distress.   His WBC count was elevated at 18, indicating infection but cultures were normal.  He went home on antibiotics.
He is feeling so much better.
Thank you to everyone who kept him in their thoughts and prayers!  And thank you to all the medical personel who cared for him along the way!

Cards

Once again, the wonderful Charla Reardon created a facebook event to ask people to send birthday cards to Ambrose.  He received 68 cards.  We want to send a special thank you to Kelley Hickey Lynch and the Armata Biance children's rosary group for the fabulous tactile cards they made for Ambrose and for all the prayers over the years!  Ambrose really enjoys touching the cards and one day made a huge SMILE while touching one of them!

SWEET ANGEL SOPHIA

Sophia's birthday and the day of her passing have come and gone once more.  On January 22, it was 6 years since she left us to become an angel.  Everyday we miss her but we feel her presence in our lives, most especially through our darling Ambrose.  My husband and I wrote messages on balloons and released them into the sky.  My husband was able to do so over Hogback mountain and watch it slowly drift away at the "100 mile view" spot.  I released mine in the backyard and am not sure it went so far, but it felt good to do.  I'm sure she got the message.

February 7 was the 10th year anniversary of her birth.  10 years!  Wow!  How our lives have changed. She was a tiny girl, but the love that radiated off of her filled the whole house.  It is hard to remember a time before she was with me.  Perhaps because she has always been with me.  I miss her so, but am so grateful that we had almost 4 glorious years with her and I know I will one day be with her again.