Sunday, January 3, 2016

Buckle In!

As I went to create this blog post, I took a look at my past posts and had to laugh...there are so many drafts that I've started and then never finished.  So let's see how long it takes this post to get's September 27th right now!

I have given lots of updates on Connor in the past if you want to read them to see where we started:

Since it's been well over a year since I've given an in depth update on him, I'll do my best to update everything (even though a lot of it is still the same).

Seizures:  Connor just had his 8th EEG (all but one were at least 6 hours). We recently weened Connor off Keppra and started a new seizure medicine called Trileptal.  He is still on Onfi.  The good news is we have seen a decrease in seizure activity and haven't noticed his traditional head jerks in a couple of days.  He has a neurology appointment next week so it'll be interesting to see what his neurologist says.  We are definitely happy with the change in seizure medication for right now.

Connor's many EEGs (there are only 7 here because one was a short EEG that we didn't take a picture of).  It's CRAZY to look back at NICU Connor and to see where we are today!

Microcephaly:  Connor obviously still has's not something that will likely ever change, but his head is about 10 cm. larger than when he was born.

Stroke:  Looking at Connor's physical movement, you would probably never know that he had a stroke.  His stroke was in the left side of his brain (likely in utero).  However, he is able to move all of his arms and legs equally (sometimes strokes can cause a lack of movement on one side of the body).  However, his seizures are likely still originating from the site of the stroke and the stroke occurred in the region of his brain that controls vision so that's another negative to this.

Opthamology:  Connor is still on 3 eye drops a day and his pressures are remaining stable.   He returns in January to recheck his pressures.  We're still not sure that he sees as he doesn't track objects, but remain optimistic that even if he can't right now, one day he may be able to.

This video is from August

Hearing:  Connor's hearing remains great...he LOVES music! He got ear tubes back in June and (knock on wood) hasn't had an ear infection since.  We are hopeful the ear tubes will help with Connor's overall health (we spent A LOT of last winter/spring sick!).

Teeth:  Connor had 4 teeth pulled back in June due to bad cavities.  We certainly miss those pearly whites, but it made more sense to pull them than to try to fill the cavitites.

 Last tooth brushing with all of his teeth.  He was not happy!

Something in my mouth feels different!
The 4 pulled teeth...they were yucky!

Airway:  Also in June, Connor had a bronchoscopy to check his airway (since he had pnemonia twice and was in respiratory distress back in October of last year).  Luckily his airway looked great.  They were also considering removing his adenoids if they were enlarged, but they were small so an adenoidectomy was not necessary.

This was one of our scariest days yet.  Connor had to be intubated due to respiratory distress last October.  Luckily, his bronchoscopy in June showed his airway looks good and normal.

Connor is now strictly fed through his feeding tube (a g-tube that connects directly into his stomach).  We were getting pressured for a while to get one, and his hospital stay last October finally convinced us to proceed with it (see pictures above...we definitely didn't want that happening again!).  Well, it took 7 tries, but we were finally able to have surgery in June.  Connor will most likely be fed this way for life.  We were very nervous about it, but the transition has been seamless and it is so easy.  We can now make sure Connor is getting all of his medicine and getting enough nutrients a day.  Since surgery back in June, he has put on 9 pounds!

One of his last pictures with no feeding tube!

Shortly after surgery.  The smiley "bandaids" are where the laparoscopic incisions they made are.

Connor can eat any time he wants...while starting the Avondale 5K or even when he sleeps!

We can easily give meds through his feeding tube as well!

We are still attempting solids with him, but his main source of nutrients comes through his feeding tube.

Connor trying avacado for the first time.  He didn't hate it!

Reflux:  Back in March, Connor was admitted for a pH probe to determine the level of acid reflux he has.  Of course, he did in fact have reflux.  This meant that when he got his feeding tube, he would need a Nissen fundoplication, which means they essentially wrap the top of his stomach to his esophogus and stitch them together.  This helps with reflux, but it also makes it difficult for Connor to throw up (although he has already proven that he still can!).  

Pulmonary Hypertension:  Last October when Connor was in the PICU for respiratory distress, he had a heart echo done.  We were told if they hadn't known that he had a history of pulmonary hypertension, they would have thought it was a regular heart with normal pressures.  While Connor was sedated for surgery back in June, he had a repeat echo done and everything looked good.  We decided at his cardiology appointment later that month to decrease his medication and see how he does with that.  He is now only on medication once a day (as opposed to the 3x a day he started off on).  We hope that this has resolved itself and we can cross this medication off our list.   

I had a good cardiology appointment, even if my face doesn't show it!

Nephrology:  I took Connor to a nephrologist appointment the week of Thanksgiving hoping it would be a specialist we could cross off.  Instead, we seemed to dig ourselves deeper.  Prior to the appointment, Connor had a renal ultrasound and the doctor noticed that he was retaining a little bit of fluid in his kidneys.  She said it could be because that's just the way he is, or it could be a sign of potential UTIs.  They had already taken a urine sample and when it looked "dirty," they had to catheterize him to get a sterile sample to test for a UTI.  As it turns out, he had a UTI, but had no fever or anything.  It was really bizarre because without this appointment, we never would have known.  
     So Connor got on an antibiotic for the UTI, but would need an additional test to determine if he had reflux from his bladder into his kidneys.  So the week of Christmas, they re-cathed him, took x-rays, and filled his bladder with contrast dye (while taking x-rays).  Then, once he emptied his bladder, more x-rays were taken.  The good news is that Connor had no reflux back into his kidneys!  This meant that the UTI he had was more of a freak incident.

No urinary tract reflux...still has acid reflux though!

Toes:  Connor's toes are still cute little nubs...that's definitely not going to change.  He won't magically grow bones in them to produce definitive toes.  His toes are actually what encouraged doctors to think there was something genetic going on.  

Genetics:  I think I've already addressed this before, but we all sent blood samples off to GeneDX where they did a whole exome sequencing.  Only one marker (?) came back that he got from me (go figure), but Matt was not a carrier and the symptoms didn't match Connor (for example: macrocephaly was a symptom of it, but Connor has microcephaly).  When Matt called me with the results, I actually cried because I didn't want to hear about life span associated with various syndromes (although his neurologist has indicated a possible life span of 10 years).  So right now, we say he has Connor syndrome!

Personality:  Connor is a really happy kid when he's feeling good and people aren't messing with him!  He has truly come a LONG way from his early days.  Connor loves playing rough (wrestling with daddy), music, and playing on his mat.

Sleep:  This is a very touchy subject and I'm not going to say a whole lot on this.  Let's put it this way:  he's sleeping better than he did when he was a newborn, but is still not a typical sleeper.  He'll give us several nights of fairly decent sleep and then go back to not sleeping well.  We are *thrilled* if he sleeps until 4:00.  That's a good night for us.  Recently, he's been waking up in the 2:00 hour.  Wahh!!!

My name is Connor and I've been known to fall asleep in my big boy chair during a feed because I decided I had to wake up really early and I'm still so tired!

In order for us to survive function, Matt and I have a whole system worked out.  For example, tonight, I will go to bed early and Matt will stay up and finish Connor's last feed.  Whenever Connor wakes up tomorrow, I will get up with him.  Then tomorrow evening, it's the opposite:  Matt goes to bed early and I stay up and finish his feed.  Needless to say our weekends are spent napping and catching up on sleep.

I think that's about it.  The biggest update for us this past year was the g-tube.  It has really been a game changer in our house and I'm so glad we finally decided to go for it.  Connor has been doing really well since he got in June.  We are keeping our fingers crossed to have a better winter/spring than last year!