Major Appointment Checkin

We just had a full day of regularly scheduled followups at Children's on 09/21/15.  

Ethan is doing very well, but the direction in terms of his future care has changed somewhat.  There has been a paradigm shift in how they treat children with CFM and Treacher Collins Syndrome (a related syndrome but *not* what Ethan has).  

The belief in the past was that you treated the hypoplasia in the mandible with the distraction surgeries.  However, in children with these two conditions it was found that about 3-4 years after the surgery, it often appeared as if nothing was done and it was a wasted surgery.  The old paradigm was to distract the lower jaw so that it would match the upper jaw.  However, since the benefits seemed to disappear after about 3-4 years, the distraction would have to be repeated (sometimes as many as 3-4 times depending on the case).  

The new belief is that in many of these cases, the best course of action is to basically 'reset' the entire jaw - both upper and lower - with a very large, major surgery that involved distracting both the upper and lower jaws at the same time as well as moving the entire jaw structure at the same time.  This is a much, much larger deal than the old surgery of distracting just the lower jaw.  It involves about a 2 week stay in the hospital after the initial surgery (part of it in the ICU) with the entire jaw being wired shut for about a month.  Feeding is done either in a G-Tube for children that already have a G-Tube, or a feeding tube through the nose.  Once the first month is done, children can begin feeding orally but because of the nature of the surgery, the distraction devices are all external and the child has to wear the whole halo/head harness apparatus.  This stays on for about another 2 months.  

The reason you distract the upper jaw is that, really, it also is smaller than it should be.  But under the old paradigm, you distract the lower to match the upper.  In the new paradigm, you distract both to the length both should actually be.  In reality, both Ethan's upper and lower jaw suffer from hypoplasia.  It is just that the lower suffers more.  

It is currently unknown if Ethan will undergo this surgery.  He will *not* undergo the normal distraction surgery that, up to now, we thought and were told he would go through.  Next year he will undergo another sleep study.  Depending on the results, he may then undergo an CT scan.  Based on the data, it will then be determined if he is a candidate for this surgery.  The point of this surgery is NOT to fix the jaw(s) for aesthetics.  The point of this surgery is to address airway issues.  If his airways is structurally compromised (due to his anatomy and CFM), this surgery will fix that.  The normal distraction surgery could ostensibly address it as well, but the benefits disappear relatively soon and another distraction is required.  To avoid multiple surgeries, this alternative paradigm was conceived which fixes the problem once and for all.  The downside is that is is a much, much bigger deal than the older surgical method.  

Also, based on the data from the sleep study and possible CT scan, it may be determined that Ethan may only need CPAP.  It is also possible the data may simply say that nothing will be done until 18 years of age.  

At 18 - there is no escaping it - he will have major jaw surgery.  Even if he must have this new major surgery, he is still looking at major jaw surgery at 18 (even under the old paradigm he was still looking at this - this has never changed).  

However, the downside to not having the surgery is that he will go through adolescence with a short jaw and looking different than other people.  And this will only get more, and more pronounced as he grows and gets older.  

So either way, there is major suckage coming.  Either a very large and disruptive surgery about 1-3 years from now, or a possibly difficult and awkward adolescence until the jaw can be cosmetically fixed at 18.  

BTW - this new surgical paradigm was conceived and pioneered by Ethan's surgeon (the one that fixed his mouth).  He's already done this 5 times and has presented his findings to an international conference where it was apparently very well received.  Apparently even his mentor agrees it is a much better approach.  The first time he did this surgery was about 5-6 years ago.  

In other news, Ethan has been patching and wearing glasses now for about 9 months.  He had a 6 month check in with Ophthalmology.  At the check in he did very well and they cut the patching down from 4 hours a day every day to 4 hours every other day.  The doctor expects by the 1 year check in, he can probably do away with the patching but glasses will still remain for another 2 years or so. 

Ethan really resisted doing both initially and the first week was kind of rough.  It helped if I would draw something interesting on his patch.  After a couple of weeks he got used to it and now it's just an inconvenient fact of life.  

- b

Patching begins...

Ethan has started patching.  It is going pretty much as expected.  The first day he absolutely hated it.  We were at my folks place in Vancouver when we started and he complained that he wasn't having any fun anymore because of the patch.  :-( 

We found the next day that breaking it up into two 2-hour sessions worked better for him and by the fourth day, he had gotten used to it enough that his first 2-hour session went to 4 hours and we informed him - much to his exquisite delight - that he was done for the day and didn't have to do a second session. 

Dina found a blog of a father who had to patch his son and made it more 'fun' for him by drawing pictures on his patch.  Needless to say we adopted this idea the very moment and it has helped a little.  We let Ethan determine what he wants drawn and then I do it.  So far: USS Enterprise, the Enterprise insignia, a Star Wars stormtrooper...  - you can see a theme here. 

One issue we've run into is that after only 5 days is that the constant application and removal of the patch is irritating his skin.  We called this in and they suggested (of all things) rubbing some Milk of Magnesia or Malox on the area before applying the patch.  Apparently this forms a kind of barrier between the skin and the patch adhesive.  We shall see to it's efficacy.  

We also had Ethan pick out a glasses frame and they should be ready sometime this week. 

- bob

Reversion To The Mean

Considering we have gone literally years now without getting a hint of bad news from Children's I suppose something like this was overdue.  Reversion to the mean, I suppose.  

We just had a regular ophthalmology follow up at Children's this PM.  

Not so good this time.  :-(  

Ethan's right eye (the affected eye) is showing signs of becoming slightly weaker.  This means the doctor wants to start patching and eye glasses now.  Patching will be 4 hours a day, 5 days a week, for the next six months.  All four hours don't have have to be in a consecutive block.  Glasses will be full time but probably will stop after age 8.  He will have a follow up om 6 months to see if it's gotten any better and if so, the patching may stop.  Or it may not.  Depends.  But according to the doctor, whatever the outcome, worst case is that it continues until age 8.  Then he's done with everything - unless he actually has bad eye sight.  

His glasses prescription is very weak though.  -1 in the affected eye and nothing in the good eye.  

We are going to try to get the prescription filled tomorrow but it depends on if he can find a frame he likes and is comfortable.  But chances are we won't actually get the glasses until some time in the new year.  

The doctor actually gave us a choice - you can do patching for 4 hours/day for 5 days/week for six months with glasses - or you can patch full time 24/7 for 6 weeks.  But we don't think Ethan would take very well to the 2nd alternative so we opted for patching w/ glasses.  

The aim is to get the brain to use the bad eye more.  Apparently at age 8, the brain is fully trained and you don't need to do it anymore.  This is prevent amblyopia - where the brain eventually just stops using the bad eye (and you go blind in the bad eye).  

As of now, the doctor is *not* concerned because the difference between the good eye and the bad eye is so small.  But he says the time to make the corrections is now and not later.  

So glasses start as soon as we get them, and patching starts immediately (so given it's 7PM and we just got home - that probably means starting tomorrow).  

-b

"Hello again, hello. Just called to say hello. "

Yes, it's been a very long time.  Over a year.  Since this blog eventually turned into a family news blog, that function soon got supplanted by Facebook where family news and events got posted (complete with pictures!).

At this point, the blog will probably just be used for posterity and to log events relevant to Ethan's condition.

With regard to that topic - nothing has changed.  Since the last update, Ethan has been doing very well and has come out of every regular Children's followup with nothing to report except that he is coming along nicely!

And for the sake of posterity - other news that has happened in the last year+
*The boys continue with Karate.  Ethan is now a Gold stripe belt (3rd color rank for kids 7 and under at his school) and Jonathan is a Purple belt (4th color rank for kids 7-13 at his school)

*Ethan started Hockey 1.

*Jonathan has finished losing his first wave of primary teeth.

*Ethan started loosing his teeth.

*Ethan attended his first Star Trek con with us and meet several celebrities, some of whom were quite taken with him!  Jonathan opted to not go and spent the weekend at Grandma's.

*Both  boys attended their first ballet - the Nutcracker.

*Yours truly became an American citizen!

*Ethan got discharged from Speech Therapy.  He is done!


To stay more current on family events, you should friend myself and/or Dina on Facebook.

-b

Update on Ethan

Ethan  had more regularly scheduled followups at Children's  on 5/6/13.  

Everything was pretty much the same and they're happy with how he's doing.  He's still on track for major jaw surgery at about age 7-9.  However, we got more details of what this will eventually involve.  Once this starts - it will not be a good year for Ethan...  :-(  

Most likely there will be one major jaw surgery before the main one to lengthen his jaw.  This will involve taking bone and cartilage grafts from his ribs and using it to create more bone and/or joints at the joint where his jaw hinges together.  It is not known definitively if this surgery will be required until they take a CAT scan to see what they have to work with - and they will not do this until about age 7 when he's getting into the window of time where they want to do the procedure.  However, it was the doctor's opinion that he would be very surprised if Ethan did not require this surgery.  After this surgery, it will be at least 6 months (minimum) before the jaw distraction surgery can take place.  

After this surgery, the jaw distraction (jaw lengthening) can take place.  This will involve breaking the jaw in two places - one on each side - and inserting the distraction device.  Once in place, there will be one month of actually using the device (turning the screw on each device on a nightly basis to lengthen it and hence induce bone to grow in the gap).  There will be at least two visits per week to Children's during this time to monitor the bone growth.  X-rays will probably be taken at each visit.  This period could go longer than one month but the doctor estimates that amount of bone that needs to be created will take around 1 month.  After this process, the device is left in the jaw for about another 2 months.  At this point - the device is what is holding his jaw together so it is left in place while the jaw heals over the next 2 months.  

At the end of the healing period, there is the final surgery to remove the devices.  After the devices are removed, he should be healed enough that no further healing is required - essentially the day after the devices are removed he is free to eat anything and do any and all activities.  However, for the 3+ months prior (i.e. when the devices are in place) he will be on a severely restricted regimen - very soft foods only, probably very little physical activities, etc.  

It will probably be a very unpleasant year for Ethan...  :-(  They did say they will accommodate you if you want a summer time surgery so he doesn't have to go to school with the devices implanted (most parents opt for this so summer is a busy time for them in terms of this type of surgery) but given that the entire process will be a minimum of 3 months - that will be his whole summer vacation so I'm sure he will be *very* unhappy about that.  

Since it's at least 4 years away, I probably will choose to simply not think about it until then.  We do not meet w/ the plastic surgeon again for another followup for another 2+ years at this point.  So nothing will change in terms of the surgical schedule so no need to think about it.  So I'm choosing not to.  

Other news: Ethan had another regular hearing screen and the results came back the same as last time so this is good.  Essentially, his hearing is not expected to change, but as he gets older, they can get more reliable data from him and that is the reason for the periodic hearing screens.  So far, the data is fairly stable: he is giving pretty consistent data telling us what we already know.  

His otolaryngologist saw him and there was nothing new there either.  There was some talk of possibly inserting ear tubes to help drain fluid from his right ear to possibly improve the slight hearing loss he has - but given it will require general anesthesia and every time Ethan requires a general he also needs to be intubated, we all agreed to simply leave it as an option on the table and do nothing for now.  

He meets with this ophthalmologist at Children's in two weeks.  We expect to walk out of there with glasses for Ethan.  We hope to be surprised and come out w/ nothing - but we're really not counting on it.  

 

 

- bob

Emerald City Comicon 2013

So after last year, I converted Dina to Geekdom and we went to Comicon this year.  This year we had the fortune to meet Patrick Stewart (Star Trek: The Next Generation), Gillian Anderson (The X-Files), Walter Koenig (Star Trek), Billy Dee Williams (Empire Strikes Back/Return of the Jedi), Dirk Benedict (Battlestar Galactica), Wil Wheaton (Star Trek: The Next Generation), Jim Cummings (the voice of Winnie The Pooh), and Grey DeLisle (Scooby Do - the voice of Daphne). We also met (but didn't get a photograph with) Christopher Lloyd (Back To The Future/Star Trek III).  Our schedule just did not really allow for getting his photograph (well, that and the quickly dwindling amount of cash we brought - photos and signatures were cash only). 

It was an amazing weekend of geekness and waiting in lines!  From our experience last year, we knew that the more popular guests (i.e. everyone *we* wanted to see) were going to have long lines, and as such, if we didn't line up early, we either wouldn't get to see them and/or it would throw off our schedule for the rest of the day and result in our missing even more people.

As a result, Dina put a schedule together of our entire weekend and we pretty much stuck to it - except where we were running ahead of schedule and then tried to pull in events/people from the next day to free up our schedule for the upcoming day.  We were there for all three days from opening to closing (first Comicon where I did *that*) and by my reckoning, we spent probably 75% of that time in lines.

Every one of the celebrities we met were very friendly and gracious.  They took time (when they could) to say a few words to you, and actually look you in the eye (as opposed to other celebrities whom I have heard will barely acknowledge your existence).   Generally we would get our photos taken and then take those to the signings (which were scheduled later) to get them autographed.  Both Gillian Anderson and Walter Koenig actually remembered us from the earlier photograph line which was ultra cool! 

We also had a chance to meet up with an old junior high/high school friend of mine who came in from out of town for Comicon from Prince George, B.C.  This gave Dina  a chance to ask her if her husband really was as geeky as he said he was back then.  (The answer was 'yes').

If you are wondering where our two boys are/were - mom and dad came down from Vancouver for the weekend to watch them while we went and got our geek on!  Thank you mom and dad!  :-)

With Gillian Anderson

With Patrick Stewart

With Billy Dee Williams

With Walter Koenig

With Dirk Benedict

Live long and prosper - and make it so!  Frack!

- bob

"Let me explain. No - there is too much. Let me sum up"

Bonus points to anyone that can identify the quote. 

So, to sum up:
Ethan's collar bone has healed quite nicely. 
Everyone got sick from some cold virus accidentally released from some secret government lab
Halloween came and went and the boys made out like bandits
Ethan discovered he *really* likes Christmas light displays
The boys were even more jazzed up about Christmas than last year (if that was possible)
Jonathan just had his 6th birthday - how the time flies! 
Jonathan is at 7 stripes on his Karate belt.  One more stripe and then after that - he's a yellow belt! 
Spent my 11th evening with Rush! 
Saw Roger Hodgson (of Supertramp)

So, to explain:
After 2 follow up x-rays, Ethan was cleared for all activities.  We stopped using the sling after about 10 days and stopped pain meds after only about 3 days. 

This Halloween, Ethan went as a red Angry Bird and Jonathan decided to reprise his roll as Thomas The Tank Engine.  Like last year, we went to Bellevue Square Mall to do trick or treating.  The mall has been getting better at this over the years with fewer and fewer stores running out of candy.  This year, we got there around the start time and stayed for about 1.5 hours and there were still stores handing out candy.  Contrast that with the first year we went where the stores generally ran out of candy before the first hour was up.  After that, we went to our church where they were running a sort of Trick and Treat thing - kids would play games (like ball toss, etc) to get their candy.  They also ran 2 bouncy houses which Jonathan enjoyed thoroughly. 

As Christmas drew closer, we made various pilgrimages to see various Christmas light displays.  Just to give an idea of how much the boys (especially Ethan) enjoy this - there are a few houses up the street from us that do a *very* nice job of put on a light display.  Every single night that we come home (from wherever we may be), we *have* to drive by those houses to see their lights before we go to our house.  Every time.  No exceptions.  We also go see various things around town that have become a sort of tradition for us now: Bellevue Square has a nightly performance of music, live drummers, and artificial snow, the Belleuve Botanical Gardens transform their entire grounds into a huge light display, the City of Redmond hosts a night where you walk from city hall to Redmond Town Center mall along the Burke Gillman bike/jogging trail where the entire distance is lined with lights and carolers - complete with family activities at both ends of the walk, and in downtown Seattle, there is the annual lighting of the huge Christmas tree down by Westlake Center mall and the lighting of the Macy's star. 

We alternate years where we spend Christmas in Seattle or Vancouver.  This year was our year to spend Christmas in Seattle.  We then headed up to Vancouver to spend time with my parents on Boxing day (and spend a few days there).  This leads to a little confusion for the boys because they get to open presents on Christmas Eve (at an annual extended family gathering at one of Dina's aunts), then more on Christmas Day, and then more again on Boxing day on the years we go up to Vancouver.  And on years where we go up to Vancouver for Christmas Day, there will be more presents for the boys to open when we come back to Seattle.  So Jonathan had some trouble understanding that Santa was only coming once not twice - once for our house and another time for my folk's house.  And on top of that, he always asks if there will be presents if we go to someone's house because there a just so many places where they open gifts! 

Jonathan, for his birthday, will have 2 parties this year.  His first one, on his actual birthday, was a Chuck E Cheese.  We invited only the immediate family and he got to pick one friend to come.  This is becoming a favorite place to go for both boys.  Ethan proclaimed that he wanted to come to Chuck E Cheese on his birthday too!  His second party will be at the ice rink close to  our house (the Kingsgate arena).  This is because Dina 'Liked' Kingsgate Arena on Facebook and then won a free ice skating birthday party!  Since we only have to supply the cake and pizza (and there is a pizza place in the same strip mall that has $5 pizzas) and $7/skater over 10 skaters we invited his entire class, a couple of his friends and immediate family.  Anyone attending the party but not skating don't need to pay. 

In other (what I deem) interesting news:
Yours truly saw Rush for the 11th time (and counting).  This was the tour supporting their new album Clockwork Angels and in my opinion, it is the best album they've put out in probably decades. 

I also had the good fortune to see Roger Hodgson (the voice of Supertramp) for the first time.  It was a fantastic show where he played a ton of old Supertramp. 

- bob