Graduate.

Before Spencer was born, I didn't know anything about feeding tubes.  All of I knew was that old people or people in comas got them.  I never imagined that not only would we have a child with a rare genetic syndrome, but he would also require a feeding tube.  

After he was born, the main emphasis of his medical conditions was his inability to eat.  I went looking for other people for support regarding his tube.  We embraced his tube and the "tubie" culture.  I became friends with many different people because many of us had a commonality, we had a child with a feeding tube.  It didn't matter if they had Mitochondrial Disease or Down's Syndrome, we had a common bond.  

As Spencer has learned to eat, we identify him less and less as a tubie. Instead of being his lifeline, it is now just a small reminder.  However, we will always be a tubie family.  We have gone through this crazy journey, as many others have, and it will always be a part of us.  

Tomorrow, Spencer will have his feeding tube removed.  He has some type of feeding tube since he was 1 day old.  Today, he has had his gtube for 3 years, 10 months, and 12 days.  Tomorrow will be day 1 of him living life as a "tubie graduate" and the beginning of his tube being a memory.   

Cardio

The big cardiology appointment is Tuesday.  I'm nervous already.  I try not to think about big appointments or surgery until the morning of.  I can usually block it out of my mind until the doctor sits down or we walk down the hall to the OR.  This time I can't.  It's his heart.  It keeps him alive.  His broken heart has kept him alive for this long but we need to repair it.  I want to know what the plan is on Tuesday.  I don't want to have to do an internal echo or any of that.  I want them to tell me that they're going to fix it, when and how, so I can prepare myself.  I'm tired of putting it off, but part of me wants to run for the hills.

On the plus side, if there is one to heart surgery, we hopefully will find out if we can pull his tube from this appointment.  I'm excited by the prospect of him being tubeless as soon as March.  He wouldn't have a 4th "tubiversary."

 

Quick end of the year review

The year started off in us (finally) moving in to our own house.  It still is a work in progress, but it's home.  We had a lot of fond memories of our past here.  Jimmy kissed me for the first time in the kitchen, and I told me he loved me on the front steps.  Our kids will grow up here and as a family we'll make a lot great memories of our own.

Quinn Avery was born in March, and is completely typical.  She has a great personality and loves to be the center of attention.  She's in the process of learning to crawl, and can do so successfully backwards.  It took Spencer a long time, but he's finally gotten used to her and seems to enjoy a playmate. 

Spencer has come a long way.  He is learning to use his wheelchair independently and is still working on walking.  He can use a walker, but doesn't like to.  He cruises around furniture like crazy and is in everything.  He hasn't had any surgery this year.  Also, his biggest achievement is becoming independent from his feeding tube.  It's looking like in 2014 he will become tube-free.  He also started school, which he adores. 

School

Shortly after Spencer was born, the doctors told us that they didn't know if he was going to live or die.  Today was his first day of school.  He did amazing.  He didn't flip out on me when we were waiting in the hallway with all the other kids, in fact, he waved hi to either a little girl or the fish in the fish tank.  He didn't have a meltdown when all the other kids cried, even the kid that was having the king of all meltdowns.  He ate his lunch, which was lovingly made just for him, and he ate snack.  He did need his Elmo (his comfort toy), twice.  He wasn't feeling carpet time, but I think once he gets used to school he will.  He loved free time and did great with other kids crowding around him. 

The first kid to warm up to him was a little girl named Olivia.  She shared with him and they sat next to each other and just played.  The kids didn't seem to notice his chair at all and just accepted him for who he is.  One did ask why he was so quiet.  Out on the playground at recess, a couple kids asked why he had the chair, and I told them that it was because his legs didn't work that great, and that was a good enough answer for them.  I like the honesty of small children.

I hope he makes some friends, maybe even a best friend.  It would be nice to have kids that aren't related to us come over or him to have a play date.  I really hope going to school builds his limited social skills and he really starts coming out of his shell.

Overall, my baby survived his first day of school!  He is so proud.  I survived too, and I couldn't be prouder of him.

Need

Spencer no longer has oxygen equipment in the house.  It has been a long time coming, but his apnea is no longer clinically significant.  I balled my eyes out after I hung up the phone.  They came to pick up the oxygen equipment a couple weeks ago.  I've never been so happy to see something go.

Spencer is still doing awesome eating.  He is eating the majority of his meals though out the day.  He's also improving significantly with a new sippy cup.  We've been giving him a bit of pediasure.  I was super hesitant, but if he were typical and still struggling with weight gain, that's what we would end up giving him.  I have this weird no pediasure through the tube thing going on though.  We are still doing a blended diet for his feeds through his tube.  We have accomplished our goal of getting rid of at least one tube feed so he doesn't have to use his tube at school.  He will still need a water bolus, but I don't have to send blend with him.  He's really doing amazing.  There is this light at the end of the tunnel now.  Thinking of Spencer without a feeding tube scares, yet excites me.

His Michelle P waiver was finalized!  I'm getting paid 13.50 an hour to be his minion.  I'm quitting my job at Lowe's.  It was time, as Spencer needs me more (and Quinn will only be getting bigger), and if I kept working we would lose his SSI completely.  It's our backup plan in case we un-expectantly lost his waiver.  We also get 4.75 hours of respite a week.  I'm looking forward to getting to spend the weekends with our family.  With our nieces and nephews going back to school I won't get to see them during the week as much.  Plus my kids can go do stuff that usually only takes place on the weekend, like fairs and such.

Spencer is finishing up his paperwork for preschool.  He starts on September 3rd.  I'm a nervous wreck.  I have this fear that he's going to be on the bus and there will be a sub bus driver and aide and they won't know who he is and he can't tell them.  His backpack and his wheelchair both have his name on them now.

Quinn's neuro appointment was this morning.  Doc thinks it's nothing, but since her fontanelle is still open, it's easy to do an ultrasound.  It's in the morning.  She also has a touch of torticollis, which we need to do stretches for.  I apparently didn't know that babies have to practice standing up....I thought that was an older infant thing.  Spencer didn't even really try until he was over a year old.  Quinn is already doing it a lot better after we practiced earlier. 

I'm nervous about tomorrow.  I have been through hell and back with Spencer.  I expect testing and results I didn't want to hear with Spencer.  I can handle the unknown with him.  It's what we've done for the past 3 years.  With Quinn though, it's hard.  It was so strange sitting in a doctor's office with kids in wheelchairs and to have Quinn with me rather than Spencer.  I kept thinking, "We don't belong here."  She has been so normal and typical so far.  It would crush my soul if she was diagnosed with something after tomorrow...even if it's something benign.  I so desperately need her to be normal and not to worry as much as I worry with Spencer with her.  I could have handled her being born with special needs.  I can't handle thinking she's a normie and then "bam" she's not.  I need this US to come back clean and perfect tomorrow.

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.