Warning - Whiney Post!

Last week was just one of those weeks.  Really long, busy and overwhelming.  So I feel like it deserves a blog of its own…

Miss Molly & Connor last week

Monday – Busy day at the office with a dark cloud hanging out above my head for most of it – tonight we were going to have to say “good-bye” to Connor’s beloved babysitter, Molly.  I picked Connor up that night, tried to snap a cute pic of the two of them together and managed to leave without crying.  We adored Molly and knew Connor was well taken care of when he was at her house.  So it’s sad (but we are happy for her and her new vocational adventure!) and it’s overwhelming because we still don’t have a child care plan for Connor.  But some leads are coming in from friends and colleagues and so far I’ve been able to remain hopeful!

Tuesday – Working from my home office stresses me out quite a lot of the time.  Connor was home with me which just complicates matters more as most of the day I feel like I’m either being a slacker employee or a slacker mom.  All the things I should be getting done today…all the therapies and exercises I should be doing with Connor.  The afternoon brought a PT session for Connor which was good.  The PT was happy with the progress little man made while at Gramma’s and we had a really nice chat about life.  I’m so thankful for Connor’s therapists…they are such kind and wise women.  After PT Connor and I had to go get Clara as she had an emergency dentist appointment for a bruised gum.  Turned out it was nothing to worry about, but still.

Wednesday – Drove to CHOP in the morning for a Neurology appointment.  After waiting in the room for an hour, the doctor finally came in, got all the updates from the last three months and examined Connor…which all took another hour.  She recommended three new consultants to address various concerns and asked me to stick around for a little bit so the CHOP OT could see Connor.  The OT came in, took history (again) and examined Connor (again).  She and neurologist concurred that Connor needs more OT and so recommended he go to a place which is actually only 10 minutes from our house.  We have now been at CHOP for three hours, it’s 2:00 and neither Connor or I have had lunch.  So we head to cafeteria where we both eat and then Connor has a meltdown.  I guess if one of us was going to have one, it is far more socially acceptable for him to scream and cry in public.  Although at CHOP, I think it would have been ok for either of us to do it.

Back in the car for an hour to the new pediatrician’s office to get her to sign paperwork for the orthotist.  I was going to have her sign it at Connor’s 9 month check up, but I had to reschedule that so we could see the Neurologist and couldn’t get another appointment with the pediatrician until August 21.  Quite frustrated with that seeing as though he needs shots and there are things I want to talk to her about that aren’t urgent enough for a sick visit.

Thursday – Bring your baby to work day.  While this, too, tends to stress me out it wasn’t too bad due to my colleagues’ excitement in seeing Connor again.  They all swooned over him and he rather enjoyed it, smiling and babbling appropriately.  Thankful for colleagues who journey with us and are tolerant of baby noises during the work day.  Managed to get a good bit of work done and then headed home.

After dinner I had to scrub the bathroom.  I hate that job.  I hate the smell of the cleaner and I hate how no matter how hard you try, you can never get the shower clean.  I usually manage to pawn it off on Brent as he doesn’t have a sense of smell, but he managed to ignore my hints for long enough that I just did it.  Luckily, Clara thinks cleaning the shower is great fun so she helped me for a while.  I figured it was “Earth friendly” cleaner so it wasn’t going to hurt her either.  I know everyone has to clean their bathroom (or pay for someone else to do it), so you’re probably rolling your eyes and thinking “suck it up, lady” but I’m not going to.  I hate that job and will always complain when I have to do it. :)

Friday – In the car again, this time for a helmet appointment.  Connor got his head rescanned as he’s getting a new helmet. 

Called to make appointments for the new consults only to be told the next appointment isn’t for five weeks.  Now this isn’t surprising, but it just urked me.  And when I called the new OT place by us I was told they weren’t accepting new OT patients, but I could be added to the long wait list.  My other option for the additional OT is to drive to the CHOP outpatient clinic in King of Prussia, about 30 minutes away.  Not sure that is doable once a week as the craziness of my fall schedule kicks into high gear in two weeks.

Realized I'm lacking in pix with Brent, so here's one
of Daddy & Connor together watching the Olympics

So yeah…it was just one of those weeks and I wanted to whine about it.  But I’m feeling ok now. This week is already better  and on Sunday we leave for a few days at the beach which means next week will be great!

In happy news, I’ve learned the Joubert Syndrome & Related Disorders Foundation’s conference is going to be in Minneapolis next summer.  I’m hoping at least Connor and I will be able to go.  I think it will be good to connect with other JS families, hear from the doctors and other presenters and spend some time with Minneapolis friends.

Thanks for reading this whiney post.
I have a page from a phrase-a-day calendar on the bulletin board above my desk that reads "Mañana sera un dia mejor" (Tomorrow will be a better day).  A good thing to remember when a day or week feels like it's caving in on you.  Thank you, God, for rainbows and promises!

Back At It

Connor and Clara at Gramma's

Clara and Connor had a wonderful time at my mom’s house for their two week stint at Camp Gramma.  Clara worked her way through the list of 17 things she wanted to do in North Dakota (I think they got almost all of them done!).  Connor was mostly along for the ride - loving all the attention he got from sissy and Gramma, doing his exercises every day and battling a cold for several days.  Brent and I had an exhausting and amazing week in New Orleans for the ELCA Youth Gathering.  So many memories and God-sightings.

Big changes ahead for us as the woman who has taken care of Connor since my maternity leave is going back to work full time.  Connor adores Molly and she and her family have become dear to us.  We are still trying to figure out what to do with the little man during the day, but have some leads and are confident a loving person will be put in our path.

Connor’s had two major tests since my last post.  On July 9^th he saw a neuro-muscular orthopedist and had x-rays done on his spine to rule out issues that would cause the popping we’ve heard in his neck.  Thankfully the x-rays came back normal and the doctor said he wouldn’t worry about the noises.  Full steam ahead with physical therapy!

The other test was on Friday (we’d been back in the state for 36 hours, so of course it was time to go to CHOP!).  This one was a swallow test because he has been choking and coughing a good bit while swallowing his formula and drool.  They gave him Barium liquid in a bottle and watched on an x-ray video as he drank it.  What they saw is that he is aspirating a little when he swallows.  The speech pathologist that went over things with me seemed a little surprised he hadn’t had respiratory issues but figured he must doing ok.  She said he did better with thicker liquid so now we are thickening his formula with cereal in hopes that will stop some of the choking. 

And so it’s back to the daily grind for the Dean family.  Brent and I got right back to work, Clara is off to YMCA camp next week and Connor is being cute, doing exercises and going to the doctor.  Current prayer requests are for Connor’s eating techniques to improve, for a sitter to be found and for continued progress with his hands’ sensory issues.

P.S.  Had to include this pic from Connor's first time at the beach.  I thought he'd freak out from the texture of the sand, but he seemed to enjoy it!

Unconditional

Connor loves bouncing on his therapy ball!

Little man continues to make progress with his therapies.  His is holding his head up for longer periods of time now and we think has made a few efforts to bat at toys with his hands.  He tolerates the eye patch and helmet still although in this hot weather he doesn’t have the helmet on for as long during the day.  Will probably mean he has to wear it past the three months the orthotist guessed at the start, but that’s ok.

While always a content, good baby, Connor is turning into a smiley baby.  Before, we would have to work pretty hard to get a smile out of him, but now he finds the strangest things funny and turns on the charm with his therapists (I think he’s trying to get out of the exercises he doesn’t like).  Connor’s also starting to acknowledge people he knows.  It’s so amazing to finally have your baby smile at you when he sees your face in the morning or turn his head when he hears your voice.  I’ve been waiting for that for many months.

We were at the orthotist (helmet lady) the other day and there were two other babies there.   I told Clara I thought they were about Connor’s age.  Almost right away, she commented on how well they held their heads up and then insisted they were older than Connor.  We asked their mom and she said they were eight months old, just a week younger than Connor.  Then about ten minutes later when the mom put the little girl baby down to play with Clara, Clara said, “She can sit all by herself?!  Connor can’t do that!”  The mom sweetly smiled at her and said, “He will soon”.   We don’t really have close interaction with babies Connor’s age so this was one of the first times she’s interacted with another baby and noticed the differences.

Kisses from Sissy

Brent and I have been honest with Clara about Connor, but have also chosen not to tell her everything.  We answer questions when she has them and explain the obvious things (his nystagmus, eye patch, helmet) to her right away so she can respond when people ask about it.  But we haven’t told her about the internal/brain things and what all of that might mean.  She’s a worrier and I don’t want to stress her out.  It’s for selfish reasons, too.  I feel like she’s really the only one in Connor’s life who doesn’t look at him and wonder “Will he ever…”.  I would like to keep it that way for as long as possible.  She thinks he’s perfect (and, I know, she will even when she knows more) and there’s something unconditional about her relationship with him that not even I can give him.  Not sure why I feel so strongly about this…even as I write now, I tear up about it.

But after this interaction with the girl baby, I know the questions are coming soon – “Why can’t Connor do what those babies can?”  I pray for the right words when the time comes.

Next week the kids are off to “Camp Gramma” – two weeks in North Dakota with my mom while I head to New Orleans for the ELCA Youth Gathering.  Brent is joining me there for part of the time to lead our synod’s youth worship band.  I'll be away from them for eight days, but am not worried as I know they are in good hands!

Stay cool! :)

Plodding Along

New eye patch

Today Connor had an ophthalmology appointment at CHOP and his doctor said it was time to try an eye patch to strengthen his left eye.  The last couple of months, we’ve noticed his left eye drifting up and in pretty significantly. So now Connor is sporting another new apparatus for a few hours each day.

Lifting and holding his head up!

We’re currently about 36 hours into a mom-imposed helmet ban as I think he was getting a pressure sore in one spot and possibly bruising on another.  We’ll see what the orthotist says when I talk to her tomorrow. 

Connor continues to improve in many areas thanks to his therapies.  It’s slow progress…very slow.  But we try to stay encouraged that he’s moving in the right direction for the most part.  He’s doing much better holding his head up and steady and seems more interested in lifting his head when lying down.

One big frustration for me in the last two weeks is Connor’s eating.  He’s always liked his bottle and started on cereal and baby food at a normal age.  While he doesn’t have a problem getting food down, he’s doing it wrong.  He sucks the baby food down like he’s sucking on a bottle – and keeps his mouth open the whole time.  Gross and messy.  The OT is trying to correct it, but the techniques she’s given us to try don’t seem to be working.

Current prayer requests and good thoughts wishes include an appointment in two weeks with a new specialist (neuro muscular orthopedist) to make sure the popping in Connor’s neck is nothing to worry about, continued positive responses to physical therapy, progress with his feeding and that the patch does the trick to strengthen his left eye.

I Do My Own Stunts

“He’s got his crash helmet on” chuckled the parking garage attendant as he made the genius observation that my baby was indeed wearing a helmet that, yes, resembles a crash helmet.  Part of me wished I could burst into tears and make up a story that contains some horrible reason why he was forced to wear such a contraption, but I did the polite thing and laughed along.

Sporting the new cranium helmet

So yes, the most visible new addition to Connor’s journey is his cranium helmet.  Most likely due to his desire/need since birth to turn his head to the left, his head became “vaulted” on the left side.  He will have to wear the helmet for about four months to squish his head into the appropriate shape.  His misshapenness was so severe that his ears were actually offset from each other by about half an ear.  Connor is tolerating the helmet quite well.  He wears it 23 hours a day unless it is super hot and/or humid out and then I cannot bear to make him wear it all day.

Most people are very sweet and almost hesitant when asking or speaking about the helmet.  We’ve had other parents whose child has worn a helmet come up, share a little of their story and then, without fail, show off their kid’s now basketball round head.  Sometimes I go into why his helmet wearing is a little different than most (most kids wear the helmet because of a flat back of the head), but most of the time I just smile and nod.

Other medical updates in the last few weeks:

• Connor had an ultrasound of his abdomen as liver and kidney function issues often accompany Joubert Syndrome.  For the first time in what seems like forever we received good news - his liver and kidney look normal!
• Blood work is being done for genetic testing.  There are two ways JS happens - either genetically or spontaneously.  I’m hoping for Connor’s to be the spontaneous form…if it genetic that means more testing for the rest of us and could make family planning more difficult for Clara and others in our family.  And holy cow genetic testing is expensive.  Immediately did a “Thank you Lord for health insurance and medical assistance” when I opened that bill!  I’m not sure how families without health insurance or who can’t navigate the system have a special needs child - it is so expensive and daunting.



Connor after Physical Therapy

• We are now being visited by three lovely therapists - weekly Occupational and Physical Therapies and Vision Therapy every other week.  We are already seeing progress - Connor is doing MUCH better at holding his head up and a few days ago he lifted his head while being held belly down.  Yup - Brent and I both got teary eyed at that one as lifting his head while on his belly was one huge milestone we noticed early on Connor wasn’t meeting.

The biggest adjustment these last few weeks has been trying to figure out how all these ongoing therapy and doctor appointments fit into our already jam packed and very calendared lives.  So far, we’ve been able to make it work this spring and it looks like we’ll make it through the summer just fine.  I’m nervous for fall once school starts again, my job gets “fall crazy” and the possibility of Brent starting to travel for work looms. It’s just a lot.  I am so thankful that Connor has a wonderful woman (ok, really a whole additional family) to take care of him while we’re at work.  Molly (yup, that’s her name, too) is God-sent and I’m reminded of that every time she asks about an appointment or agrees to have a therapist come into her home to do a session.  God has given us what we need to do this so far and so in the moments that I feel like it’s all too much I need to remember that what is needed will continue to be provided.

Thanks for your love, prayers, well wishes, offers of help, story sharing, advice and reading.  We appreciate it! :)

Starting Out

One year and six weeks ago Brent and I got the surprise of our lives.  I had been sick for three weeks.  Jokingly, several people had asked if I was pregnant.  I laughed this off because it was not possible.  Not physically possible for me to get pregnant naturally.  But that question wouldn't get out of my head, so we swung by CVS and bought a pregnancy test.  While Brent was giving Clara her shower, I took the test.  To my amazement, there were the two blue lines.  Clear as day.  I screamed.  Brent came running.  I showed him the stick and we were both speechless.  Impossible.  Unbelievable.  Miracle.

Fast forward to October 26, 2011.  Our little man comes into the world.  I was recovering from the C-Section and didn't even know our baby had been rushed to the NICU with a pneumothorax (hole in the lung).  And so the medical carnival had begun.

At 8 days old, Connor came home.  In the hospital we noticed he had rapid eye movements and a droopy eyelid, and so we set up an appointment with a CHOP (Children's Hospital of Philadelphia) pediatric ophthalmologist. He said Connor had nystagmus (eye movements) and ptosis (eyelid droop).  There wasn't anything we could do for the eye movements, but the ptosis would require surgery sooner than later.  At seven weeks, Connor got an eye lid "tuck" so his eye would be open and his visual development would not be affected.  The surgery was a success (not perfect, but functional).  Additional doctor visits and tests could continue for his eye issues.

In the last two months we (and the doctors) have noticed developmental delays in Connor.  He didn’t roll over until he was five months old, he cannot sit up, he doesn’t reach for things or grasp toys well. The muscles in his shoulders and neck were very tight - torticollis.  We decided to enroll in our county’s early intervention program and Connor qualified for physical and visual therapy.  At about the same time, our pediatric ophthalmologist suggested that Connor’s nystagmus (his shaking eye movements) are the result  of something more and not a standalone issue as we have been thinking.  He recommended that we see a neurologist and get a brain MRI done.  We saw a pediatric neurologist at the Children’s Hospital of Philadelphia (CHOP) and Connor had the MRI on April 10.  The test showed abnormalities in the brain.  

Connor has been diagnosed with Joubert Syndrome http://jsrdf.org/ It is a rare genetic disorder in which the part of the brain called the cerebellar vermis (think cerebellum) is underdeveloped or missing completely.  In Connor’s case, his is smaller than usual.  A long and varied list of symptoms come with Joubert Syndrome and the severity ranges greatly (much like there is a spectrum that children with autism fall on).We don’t know where Connor will fall on the spectrum of JS.  There is more testing in the near future to learn as much as we can about his situation, but in reality a lot of it is wait and see.  We are confident in the care we are receiving through CHOP and are so grateful that such a fabulous facility is less than an hour from our home.

As you can imagine, this is quite a lot to digest as parents.  We are doing ok.  Some moments are better than others.  The unknown is hard.  We find comfort and strength in family, friends, your prayers and love.  Connor is a beautiful, happy baby and we remain hopeful that he will grow into a strong, healthy boy and man.  But we are realistic that that process for him will be more difficult and limited than for other children.  We thank God for his life and presence in our family. 

And so, I'm blogging.  I'm blogging because we want and need to keep our friends and family updated and we don't have the physical or emotional capacity to do it individually.  I'm blogging because writing is therapeutic.  I'm blogging because I'm terrified and overwhelmed in my role as a mother.  I'm blogging because we have a miracle on our hands and I want to share him with the world.  I'm blogging because I hope it helps us on this journey and just maybe will help someone else on theirs.

So that's where we're at.  I'm not committing to writing daily or weekly or monthly.  Just when I want to. :)  Thank you for praying, laughing, crying and journeying with us.