What's Going On With Connor?

We've been asked this several times by people helping us with Connor.  Where to begin?!  There's so much going on it's sometimes hard for us to remember it all.  So let's start from top to bottom...

Head:  Connor has a lot going on in this area.

• Microcephaly.  Connor's head is much smaller than it should be.  Here's a diagram that compares his head size with a "normal" newborn's head size.  Because of this, he'll likely have problems with his cognitive abilities and neurological functions.  Looking at him I think his head looks proportionate, but when you look at other babies you realize his head is much smaller than it should be.  As the doctors told us, his head should be the largest part of his body, but his torso is the largest part.

{source}

• Seizures:  Connor had his first seizure when he was only 4 days old.  Luckily Matt and I weren't there to witness it because it was a tonic-clonic seizure where his body jerks uncontrollably.  We were told this seizure lasted about 5 minutes.  Needless to say he was immediately transferred from the Level 2 nursery at MUSC to the NICU.  He was hooked up to an EEG that evening to determine if he was still having seizures.  He had been "loaded up" on phenobarbital after the initial seizure and the EEG showed nothing.  Everyone hoped it was a one time thing.  This was on Wednesday and by Friday there were talks of us going home the following Monday.  On Saturday he began going apneic again (more on that later) and he was hooked up to the EEG again to determine if he was having seizures.  That evening they found he was in fact having seizures again.  Most EEGs are 24 or 48 hours.  Connor ended up being hooked up 4 days.  We were able to wean him off the phenobarbital and he is currently on Keppra to control his seizures.  We've had to up his dosage since we've been released from the hospital due to suspicious seizure activity (see video).

          In total, Connor has had 4 EEGs.  One 24 hour one in the NICU when he was 4 days old, a 4 day EEG also in the NICU during his first week, a 5 hour EEG after hospital discharge due to suspicious seizure activity, and a 20 minute EEG which serves as a baseline. 

• Stroke:  When Connor was 3 days old he had an MRI done.  We found out that Connor had suffered a stroke on the left side of his brain sometime within the previous 2 weeks (so likely in utero).  We soon discovered that his seizures were originating from the area in the brain where he had his stroke.  The neurologist told us that {generally speaking} babies brains are still forming and can easily be rewired so a stroke isn't quite as debilitating for an infant as it is for an adult.  Luckily Connor appears to be moving all limbs equally and seems to have the same amount of strength in all limbs.  We are very lucky because he could easily have trouble moving one side of his body.
• Brain Abnormalities:  The MRI also showed several other brain abnormalities.  Connor's brain stem had some issues and they thought this could be why he was going apneic.  Luckily that isn't the case because there was talk that day of his quality of life and would he survive.  A very, very dark and sad day for us as parents.  Also part of his brain that should be rough and have ridges is smoother than it should be.  He will have another MRI sometime this summer to hopefully provide some more answers for us.
• Eyes:  Connor's left eye (cornea) did not develop normally.  His eye is cloudy and we've been told that although it's still too early to know what his vision will be like, it will likely range from limited peripheral vision to blindness in his left eye. Right now the right eye looks perfect developmental wise, but we won't know how his vision will be until they can really take a good look at the back of his eye.  This will be done this summer in conjunction with the MRI because he will need to be put under anesthesia.  Two opthamologists have suggested his left eye has Peter's anomaly.  With everything else going on with Connor it was thought he might have Peter's syndrome which is extremely rare.  We won't know about that until our genetic tests come back, but the on a recent eye visit the opthamologist thought it might not be Peter's so we continue to wait.  Right now we are working on getting eye contact from Connor.  He reacts to light so that's gotta be a good sign, right?!

 

• Recessed Chin:  Connor's little chin, which I think is just SO cute is recessed and he has an overbite. 

 

Pulmonary Hypertension:  Connor was given an echocardiogram while in the hospital and it showed a few things that are normal within the first few days of life.  Literally the day before we were set to be discharged from the hospital, they did the follow-up echo expecting everything to look normal.  Turns out Connor has pulmonary hypertension which means that the blood vessels that carry blood to the lungs are working too hard.  We had to stay 3 extra days in the hospital and come home on oxygen and medication.  Connor is still on the medication and thankfully got off the oxygen recently.  The last doctor we met with said it will likely resolve over time and on medication.  He also made us feel better because there had been a mention of doing a heart catheter with him where they would put him to sleep and run a catheter through a vein to his heart and really test the pressure and also test which medications he responded best to.  This last doctor said he's never felt the need to do that with an infant so that made us feel much better!

Clinodactyly:  Connor's pinky finger on both hands are bent outwards.  This is actually something he appears to get from my dad!

Hydronephrosis:  A renal ultrasound done in the hospital revealed that Connor's kidney's weren't draining properly.  In our discharge paperwork it looked like they did a repeat renal ultrasound and it had improved.  We're hoping it improves, but were told it's an easy fix with antibiotics, but I've heard surgery could even be possible! :/  We'll have a follow up appointment with Nephrology this summer and should get some answers then.

Toes/Feet:  Connor was born without bones in his toes.  An x-ray at MUSC also found that he was missing some bones in one of his feet.  We've been told by several doctors that Connor will likely be able to walk so that's a huge relief to us!  Since it is all Connor will know, he should be able to adapt to walking without toes.  They described his toes as being made of cartilage like material.  There may be future concerns about what to do with his toes since you wouldn't want them to get ripped off if they got caught on something.

There are some things we can cross off and we're done with {hopefully for good}!

Apnea:  The morning after Connor's MRI, he wouldn't eat and his oxygen levels kept dropping.  Little did we know, this was all a precursor to his seizure that afternoon.  Once in the NICU, he didn't seem to have anymore issues.  That is until Saturday.  Saturday his oxygen levels kept dropping again.  We had a feeling the seizures had returned and Connor was put on the EEG that evening.  It wasn't even midnight when they called to tell us he was in fact having seizures.  While on the EEG for those 4 days, his oxygen levels continued to fluctuate and he was going apneic, meaning he would stop breathing.  There was even a time where he turned blue...thankfully Matt and I weren't there!  Since he was on seizure medication, he wasn't showing signs of seizures, and the EEG wasn't really picking any up, we had a serious conversation with the neonatologist.  Matt asked him point blank if there was a chance Connor could die from this.  He said yes and I thought I was going to die.  It's definitely not something a parent wants to hear.  The reason he said yes was because we still weren't 100% sure that the apneic events were related to the seizures.  If they weren't, it was likely due to his brain stem abnormalities and it would be an issue of his brain not telling himself to breathe.  If that was the case, then we would be faced with that ethical question of should we keep him alive solely for our benefit when his body seemed to indicate otherwise.  Thankfully we met with the neurologist a couple of hours later and he backed us off that cliff.  He said he had seen kids much worse off than Connor whose parents never had to make that decision.  Ultimately once Connor was on Keppra for his seizures, the apnic events and oxygen saturation problems stopped...thank goodness!

Apnea Monitor:  Connor came home on an apnea monitor and wore it 24/7 for a month.  After a month, he only wore it when he slept.  Finally after 2 months he was totally off it!  The main reason he was on it was because he was weaned off the phenobarbital and they wanted to make sure he wasn't going to stop breathing once it was out of his system.  Honestly, he probably could've come off the apnea monitor after the first month.  I spoke with representative from the hospital and she said it would be up to cardiology to remove it and I reminded her he wasn't on it for the pulmonary hypertension, but because of the seizures.  She then said to just keep it on him when he was out of sight so that was pretty much only when he was sleeping.

 

This picture won't rotate for some reason and it drives me crazy!

 

Oxygen:  I swear, we didn't think we'd ever get off the oxygen.  I know 3 months isn't that long in the scheme of things, but when you're sleeping on the couch separated from your significant other, it stinks!  Connor wasn't on oxygen because of breathing issues.  He actually needed it to boost the medication he was on for his pulmonary hypertension.  It was so frustrating because we just felt if the medication was supposed to be so great, it should help it on it's own.  Finally on May 15 we went to the cardiologist (again!) and this time the cardiologist said we could get off the oxygen.  We were ecstatic!  I told him he was glowing more than the anesthesiologist was during labor!

 Connor was not a fan of his oxygen...

Blood Sugar:  Connor's blood sugar was low during his first week.  He got his heel pricked before and after each feed.  As I'm sure you can imagine, he did not love this.  I can't say I blame him.  Luckily it is done!

Other Blood Issues:  We had to go to hematology for a follow up appointment and we were cleared!  I believe there were some concerns about blood clotting, but all is well.

Fever:  Connor was admitted to MUSC when he was only 2 days old.  That evening, we found out he had a fever, which is extremely uncommon for a baby that young.  He was quarantined off in case it was an infection and we had to put on gloves and a gown every time we went in to see him.  It was SO sad!  Luckily, it wasn't an infection and we were able to stop wearing the gowns and gloves.

So, that's where we stand now.  Connor is doing great, all things considered.  We love this little munchkin SO much!  It's hard to believe that one year ago today we started this journey with a little pink line...