An open letter to Dude's teachers

To Spencer’s Future Teachers, 

      Congratulations!  You have the pleasure of teaching my amazingly awesome, super adorable, and absolutely amazing child.  I’m sure you’ve heard a lot about him.  His first week of school will be hell, for all involved, due to all the changes.   Don’t let this deter you.    Don’t be afraid of him.  He’s actually freakishly well behaved and a pretty mellow guy once he gets in a routine.   His father and I are also great resources to help you teach our child, so please utilize us as much as you absolutely need to, as in the end we both want the same things…for Spencer to be the best Spencer he can be and for him to love school.  

I’m sure you’ve read his IEP and his lengthy list of diagnoses.  The thing to remember about Say Barber Biesecker Syndrome, or Genitopatellar Syndrome, or any of the other thirty of so diagnoses Spencer has is that they do not define who he is.  Spencer is well behaved, brave, a talented musician (although I’m a little biased), funny, compassionate, and a great big brother whether he wants to admit it or not.   He however is not, hypotonia, hearing impairment, cognitive delay, or arthogryposis.

Sometimes it’s easy to forget that he’s three and a half and not a baby.  Please don’t treat him like one, because the second you do, he will eat you alive.  He knows how to play the game of letting people underestimate him.   Make him do things he doesn’t want to do.  He’ll cry a bit.  He’ll either get over it or have a complete and utter meltdown, but at least he tried it and we can try it again or adapt for the next time.    The only way he will grow is if we challenge him.  

My biggest fear with Spencer starting school is the other children.  There are safety concerns, naturally, but I am more concerned about how he may be treated.   Don’t let Spencer isolate himself.  He’ll try to.  He isn’t a huge fan of other children and will often cry when they approach him.  My biggest hope for school is that he will get over that and make some friends, and If he is sheltered from them he won’t.   Kids don’t judge at this age and will hopefully see a buddy, not a wheelchair or a feeding tube.  

My biggest piece of advice?  Don’t panic.  I have kept this child alive for three and a half years with no formal medical training (I actually have an education degree).  You’ll be fine.  Teach him.

Sincerely,

Spencer’s Mom 

I never imagined my life would revolve around poop and pee

So a series of mixed updates.

So Spencer's bladder still sucks...but doesn't suck any worse.  He was super backed up with poo, which may be limiting his bladder function as well.  We never realized it, but now have him on a pretty strict clean out regime.  My theory is while he poops, his body can't get all of it out at once, so he stays chronically backed up.  We'll see what GI says when we go back in August.  As of now, he's getting a cap full of miralax at night, which seems to be helping a little too much.  I might back it up a bit and add some prunes in the morning.  It's very much a game that we play with his guts. 

Spencer is eating like a champ!  He's been eating enough a couple times a day to cut out the feeds.  We still have to do water boluses when he mouth eats because he still has a hard time with liquids.  Our goal was to get him off one tube feed a day before school started, and we've exceeded that.  The new goal is to get him to eat all his meals orally, and supplement calories where needed.   Hopefully he starts to do liquids orally and can keep his weight up, and eventually get rid of his tube.  It's like there is a light at the end of the tunnel now.  It's amazing what progress he's done in just a month.  I also suspect he will have to be feeding himself.  He loves avocado, which is high in calories and great for him.  I am scared about the potential of no longer having his tube.  His feeding tube, for so long, was our normal.  We still, at minimum, have another year of it, so no fretting yet.  It would be awesome to be able to have it out by the time he started first grade.

Dude also graduated from pulmonology!  His sleep study came back with no clinically significant findings.  As soon as I got word, I took all the oxygen supplies out of his medical cabinet and took it straight to the outside trash can.  I'm so excited to be done with it.  It has always been hanging over our head.  They haven't come to pick up the equipment yet, but I will be quite happy when they do.

Spencer's Michelle P Waiver was also approved.  We are waiting for a couple last bits of paperwork and then we can start using it.  The first thing I'm getting is solid wheelchair wheels.  Spencer got a flat and we've been without for a few days.  Hopefully the replacement inner tube comes tomorrow.

Quinn has a giant head.  Her head size went from 30th percentile to 94th in two months.  Her peds is mildly concerned and wants her to see a neurologist just to make sure everything is ok.  Of course I'm a wreck about it and she can't get in to neuro for another month and week.  It figures that out of all the specialists Spencer sees, neurology is the one department we do not see so I have no strings I can pull.  She's going to see a neurologist down here and if anything needs to be done, I'll transfer her to Cincinnati.  We suspect she is fine.  She's meeting or exceeding all her milestones and doesn't show signs of hydrocephalus.  Giant heads runs in Jimmy's family, and Spencer had a similar head growth spurt around the same age. 

Nervous.

Tomorrow we head back to Cincinnati for Spencer's Urodynamics study.  I'm a nervous wreck about all of it.  It's a hard test for anyone to go through, but even more so for a child.  Essentially they are going to fill his bladder with saline to measure the pressure inside.  Then after that hot mess he has to have a renal ultrasound.  Then they will try to get him to pee on xray.  It's a fairly miserable experience for all involved.  Jimmy is going to take the ultrasound, since I absolutely hate doing them.  I hate doing all of it, but I hate having to position him and try to get him to relax.  I'll take the other two tests.  Luckily, we won't have to wait long for the results because his urology appointment is at 2 on the same day.  Worst case scenario he has kidney damage and we have to start cathing him.   

Dr. Hopkin gave us some hope.  Spencer's bladder issues were caused by some nerve damage to his spinal cord.  There is a small chance that some of the cells may regenerate and could help bladder function.  I'm going to pick Dr. Alam's (his urologists) brain while we are in clinic about this.  The last thing I want to do is start cathing Spencer.  Usually once you start, you always have to do it.  He is 3 and would have to have this done for the rest of his life.  His prognosis is good, so cathing would be something he would still have to do as an adult. 

Screw it, we're just calling it Spencer Syndrome

Just got back from an overnight stay in Cincinnati.  Spencer had some labs drawn, a ortho followup, a visit with Trauma Services, a sleep study, and a genetics follow up.  It's been an exhausting couple of days and we get to go back Sunday to do more testing and appointments.  Joy. 

Let's start at the beginning....Spencer had his tests done for school....Hemoglobin and Lead....His hemoglobin is 15.3 and his Hematocrit is 43.7.  Both are high, but if I remember right it's common with kids with heart or airway problems.  I'll ask his pulmonary doc when we are there in a couple weeks.  He also had his lead level tested which was super low, something I've been super paranoid about since moving into our ancient house.  He also had a renal profile and blood gas done, which both look pretty good as well.

Then came the Trauma Services appointment.  Basically we were there discussing whether or not Spencer needs a special needs car seat since he's outgrowing his current one.  He does not and we scored a free new combination booster seat. 

Then we went to see Dr. Tamai, his foot orthopedic doc, for a club foot followup.  There was a kid there at the same time we were that was named Nicholas Spencer, which gave us all a good laugh.  Anyhow, his feet look great and everything fits pretty good.  We did discuss a potential foot surgery in the next year or two, if his right ankle doesn't straighten up.  Either we would release a tendon or put a pin in the growth plate.  We'll cross that bridge when we get to it. 

Sleep study blew.  First we had to wait forever for his first dose of Chloral Hydrate to show up.  I wouldn't let them touch him until he went to sleep.  He woke up at some point during the hookup and went in to sensory meltdown.  I requested the second dose of medicine, which you would think they would have sent up to the floor already considering it was such a pain in the ass to get the first one.  That took another hour.  You could just see the RTs getting antsy because of time.  It took me forever to calm him down and I finally did shortly before the medicine came in.  I was about to cut someone's throat.  Anyhow, got him back asleep and hooked up finally about 11.  He slept most of the night and only woke up to grump a few times.  We should have the results in a couple of weeks.  If this went well, it should be our last one and we can get rid of the oxygen equipment in the house. 

Finally, genetics.  We "graduated" to once a year appointments today.  We discussed Spencer's gene mutation on Chromosome 10q22KAT6B.  There is another syndrome that shares some of the gene mutations with Ohdo syndrome, called Genitopatellar syndrome.  Spencer shows characteristics of both syndrome and could very well have both or something that combines the two.  The researched in the UK has seen several people with an overlap.  Our geneticists at Cincinnati are going back again and looking at his lab results from the Ohdo testing to see what they can find.  Spencer essentially is the poster child for both syndromes.  It will be interesting to see how this turns out.  I've decided if he has something they've decided new entirely, I'm demanding it be named after him. 

Struggling.

Spencer has been home from the NICU for three years.  As his gift, we bought him a small helicopter, that he loves.  We plan on going to the pound later in the month, after our Cincinnati appointments coming up, and let him pick out a pet if he chooses.

I've been really struggling with Spencer's disabilities lately.  The hardest one is that he can't talk, and he likely will always struggle to use speech.  I would kill to hear him ask me a question, or tell me how he's feeling.  I know he'll talk...whether it be with a device or not, but he should be talking my ear off now and it's hard that he's not.  It's hard that he doesn't have a voice.

Quinn is doing awesome.  Spencer's even starting to warm up to her and steals her toys on occasion.  She is just starting to lift her head up during belly time (which she hasn't had a ton of).  She's "talking" up a storm.  Jimmy started cooing, and Quinn did it back.  They took turns in doing it for a while and even Spencer did it once or twice. 

He is doing awesome with his eating.  He still doesn't know quite what to do with ground beef yet.  He loved soft tacos, but struggled to get the meat down too.  He sloshes the soft taco shell in his mouth.   Next time I'm going to do hard shell ones and that should help him chew.  He ate over half a pita pizza pocket the other day.  He'll be ready for a happy meal in no time.

Clean Plate

So Jimmy and I decided last night that we would just start sitting Spencer down with a plate of food and see what he would do. Tonight, I made Dude and I plates and we sat down and had dinner together. I fed him to avoid a huge mess. He let me feed him with a baby spork, something he hasn't let me do in forever. He ate everything on his plate. EVERYTHING. He has never cleaned a plate before...ever. He loved baked potato. He ate chicken with BBQ sauce, asparagus tips, and carrots too! Of course his servings were probably 1/3 of a kid his age normally would get, but still, this is huge!

Bittersweet

Spencer at nearly a half of a peanut butter sandwich today.  We then had a clogged tubie after I did his tube feed, which thankfully I got unclogged in a last ditch effort before I changed his tube.  Never a dull moment.

Spencer has been incredibly vocal lately.  He said his I love you to me again last night.  He has also been saying no a lot, which is getting a lot clearer.  He is responding to me more when I talk to him.  There are still times where he just ignores me too:)

He still isn't a huge fan of Quinn.  I occasionally catch him smiling at her, and then stops when he sees me smiling at him.  Quinn started smiling about two weeks ago.  Today Jimmy said she gave him a big old "spencer" smile.  I can't wait until she starts giggling.

She grasped onto a rattle today.  I started crying.  Spencer didn't grasp onto a rattle until at least six months.  When he was her age, his hands were still in splints a huge chunk of the day.  It's bittersweet.  I'm excited by all the cool stuff Quinn is doing, but yet saddened that Spencer had to fight so hard to be able to do the same things.