Moving over the CVR Mountain!

I’m five months behind in posting, but I hope to catch up now that I am on leave with baby #2.

So this has been in the making for over a year.  I read about it.  I dreamed about it.  I stayed up countless nights worrying about it.  AND finally, on Tuesday, October 28^th  at 7:30am, Nic had her cranial vault reduction surgery.  I have talked about it many times, but for those that need a slight refresher course here goes:  Nic’s condition was found in utero on her 18-week sonogram.  At the time, her ventricles put her in the category of severe ventriculomegaly.  By the time we saw a specialist at 21 weeks, her ventricles had grown, changing her diagnosis to severe Hydrocephalus.  We watched her condition grow asymmetrically until she was born at 37 weeks.  At 32 weeks, she consulted with a NSG at Children’s Mercy who found an interhemispheric cyst.  This would become Nic’s primary diagnosis as it formed first and cause all of Nic’s other conditions (PMG, CVI, Chiari Malformation, etc.).   All of these conditions working together created one, very obvious problem, Nic’s head was growing to accommodate all of the fluid.  When Nic was born, she had a head circumference of 58 cm; she had severe Macrocephaly (http://www.healthofchildren.com/M/Macrocephaly.html). 

            Having an adult-sized on an infant body causes a number of issues.  Nursing, feeding, positioning and handling all become difficult.  For Nic, we found that after working with a private and federally funded PT twice a week for a year, she still did not have head control.  She could not left her head at tummy time, could not hold her head up independently in supported seating, could not roll over, etc.  Her head acted as an anchor.  She could at 8 months turn her head 180 degrees when in a supine position; this is it. 

           

            CVR is a surgery in which a team of surgeons cut down skull plates and put them back together, thus reducing the skull cavity size.  While this surgery was “elective”, it was decided that Nic faced a quality of life issue.  We didn’t know if she had the neurological ability to hold up her head.  As her mother, I feel she does.  She wants to move; she tries to move.  She just couldn’t move!  And how could she was a tiny body and a head my size?!!!  We knew without the surgery, she probably wouldn’t be able to gain head control until around five, when her body grew into a size more proportional with her head.  As a special educator, I knew what that would mean for Nicolette in terms of her developmental delay.  So much development in infancy is linked to gross motor development.  What kind of parents would we be if we allowed her to have such a huge delay?   If we could do something (like reducing her head size in CM and weight) and elect not to?

Now I’m going to be honest here. No one births a child and hopes for a disability.  No one hopes to see their precious child start the world “behind” or with more-obvious obstacles then his or her peers.  In my opinion, the world is cruel to conditions it collectively does not understand, and I found it devastating to learn that MY child would have to work harder and longer to try to be close to “typical”.  Now, there is a flipside to being a parent of a child with special needs that Erma Bombeck illustrates nicely in her poem “The special Mother” (http://www.our-kids.org/archives/Special_mother.html), but that’s a different story learned later as your child grows.  As a parent, you search for how to cope with loss, that deviation from dream to reality.  For me, it was to be as educated as possible, and to use that education to give Nic EVERY opportunity to “close the gap”, every opportunity for Nic to thrive.  I have read every article I can find on each of Nic’s conditions, I have consulted with “the best” provider(s) suited for Nic’s needs (even skyping a vision specialist in Europe at 3 am to accommodate a 7-hour time difference), I have joined countless support groups, and I have harassed every specialist that will give me the time of day.  I’m not the most educated Hydro parent, but I guarantee I knew them.  Why does this matter?  Because in doing these things, I have found peace.  These resources led me to Duke for Nic’s cord blood infusion, pointed me in the right directions for services to consider (honestly, I would have dropped the ball on vision services if a very dear hydro parent hadn’t said something), and prepared me when Nic was very young for the need (YES THE NEED) for CVR.  Nic could have died during the procedure; it’s invasive and bloody but I would have still had immense peace knowing that it was something she needed in order to thrive.

Nic’s CVR surgery took 8.5 hours and required two blood transfusions.  I prepared to be at the hospital for 10-14 days given Nic’s history of needing a little more time to recover post op and her history of having incubation/excavation issues.  Amazingly, Nic sailed through recovery.  We came up Monday for her pre-operative appointment, had surgery Monday (starting at 7:30am), stayed in the PICU (for excavation issues as expected) for three days, and went to the neurosurgery floor for 3 days.  We went home Saturday morning.  

Nic took two weeks to recover from post-operative pain at home and about a month to get back to her cheery self.  Holding her became the greatest challenge as the skull plates are put back together with brackets that irritate her skull and stick out notably, but we figured it out.  Nic’s head went from adult-sized (58 cm head circumference) to toddler-sized (55 cm HC).  I know the change in HC is small, but that is due to the position of HC measurement.  In reality, the team took the whole back part of her head off, a 45% reduction in head size.

Goodbye Back Part of my Skull!

 

Now, three months post-op, Nic still cannot hold up her head, but she still has a 3-year-old head on an 18-month body.  She can hold her head up independently when supported in a seated position.  She is looking side-to-said AND up-and-down when in a seated and supine position.  She is rolling from side to back.  She is moving her head, arms and legs during tummy time, though she hasn’t quite figured out how to coordinate this movements to roll from prone to supine (tummy to back).  She tolerates being held upright for much longer periods of time and more often during the head. 

Goodbye Neck Pillow!

More progress to follow in her 18-month post!

Nic Spends the Remainder of Summer at CMH

I'm really behind on this.  Late May we noticed that Nicolette did not want us to hold her anymore.  What first seemed like potential soreness from some really rigorous PT ball work, turn into serious concern.  Everything we picked her up, our sweet, happy baby started to scream and cry.  I took her into the doctor's office multiple times and even demanded to see an Audiologist convinced it was an ear issue (infection or fluid) leaving each the with zero answers.  Mid July rolled around and we began to see a complete decline in eating progress.  It was like Nic had forgot how to eat all solid foods, and sure enough one week later, she stopped bottle-feeding.  What little she did eat, she failed to keep in her stomach.  Perplexed, we packed her in the car and headed to CMH.

The next 3.5 weeks were a blur.  I can remember how rudely I was treated the first day when I demanded an ENT doctor come look at my child’s right ear or when I demanded the resident give her a strep test.  I remember insisting of daily visits from the music therapy team because that seemed to guarantee at least one laugh a day from Nic.  I can tell you that I slept in the hospital every night with Nic watching her get worse and worse, struggle to breath, and how horrid it felt when no one was able to tell me why. I remember difficult decisions and being asked for permission to treat theoretical medical conditions.  I remember what the hallways looked like when I personally walked next to her as we were moved to the PICU.  I can tell you my milk supply cut in half while in the PICU.  I can remember the need to heavily spellcheck a despite email to two hydro mothers asking for help when her shunt was ruled out for the 5^th time yet Nic could no longer breathe on her own.  I can tell you I talked to 34 different specialists and 62 hospital staff members.  And I can still remember how time froze when Nic coded, the small PICU room filled up with specialists and doctors, and how hard it was to listen to the head of the PICU when she told me to call my family because Nicolette would not make it.  I remember the look on her face, she felt as helpless as I did.... and in a moment, I honestly hated her for it.  I can remember how calm I was asking her neurosurgeon if he agreed that she would indeed die; how carefully I listen to him tell me that that he was optimistic but how critical the next step was because the margin for error was gone (…and oddly how simultaneously I thought I was going to have to write the neurology team an apology for ripping them when they told me she wouldn’t live to be over a year old).  I remember how completely alone I felt hearing all of this information, and how determined I was to make sure Nic never felt the way I did.  I remember that even after I was told all of this,  I still demanded music therapy came daily because, maybe, she could still smile on the inside when they played. And finally, I remember how happy and infuriated I was when our NSG (thank god he stayed on her case after he cleared her shunt!), discovered via MRI a huge abscess deep down her throat on-you guessed it-the right side.  And still, months later, I still feel so humbled that I have a daughter that can brave 4 neurosurgeries and a month of medical incompetence.  Here is a random part of the story, but a cherry on the crap cake, what was the abscess full of?.... Strep….. go figure.

I’m not writing this post for pity.  I’m writing for the opposite reason.  If I have learned one thing (and I can assure you, I’ve learned many things) from my child, it’s to trust in myself as a parent.  I had the wrong part of the ENT specialty, but I KNEW she needed an ENT to fix her issue.  I KNEW in late June that something was wrong with my child, that is wasn’t just a sore muscle from a heavy PT session.  Before Nic went down for her life-saving MRI, I had completed transfer paperwork to move her to Boston, because I KNEW she wasn’t getting the care she needed at children’s mercy.  I knew that regardless of how that hospital story ended (with her and me leaving or me leaving alone) that I was so blessed to have her.  AND I KNEW I was meant to be her mom.

I want to say thank you.  Thank you, the reader, for sharing in our journey.   Thank you for allowing me to discuss our story openly, even when the story is scary.  Thank you to her Neurosurgeon who has saved her life multiple times now.  Thank you to the group of hydro parents that checked up on Nic everyday offering experience, sympathy and advice.  Thank you to our family that dropped everything to be with Nic when we needed them most.  They made it possible for Nic to never have to be alone and for me to still be able to eat and shower.  Thank you to everyone that came when we thought she was going to pass.  And most importantly, thank you to Nic for never giving up, because you made me refuse to give up.

Sip and See

An important even happened on the 26th.  We were finally able to have Nic's Sip and See.  For those of you that are unfamiliar, a Sip and See is a party for the adults of the new parents, a time to come have food, drinks and see Nicolette.  They tend to be done about a month after birth, but since Nic spent that time in and out of the hospital, we thought it was best to wait.

We themed the party after the Dr. Seuss book Oh the Places You'll go! switch seemed fitting given everything she had been through and the long road ahead.  Many, many guest came to see this amazing baby.  Here is what Nic thought of the event.

I wish I'd have taken more pictures, but I was so happy to finally be around a lot of people again, I really forgot.  I can assure you, there was a TON of food and some delicious drinks!  Why such a big 6 month party?  We decided Nic's one year birthday was  going to be just for us to take a special trip to Kansas City.  Also, I WILL be the mom that throw HUGE parties for Nic during the first couple years of her life.  One, she wasn't supposed to be here.  That alone seems worth celebrating.  Two, I know NIc will struggle.  I know she if different, and my husband and I are well aware that there may be a day in which her same-aged peers stops coming to her parties.  That day will hurt.  I want us all to have these moments to look back on before we move in taking trips or special outings on Nic's special day.

All decorations were in theme with the book.  Cevin hung all the parachutes I made out of decorative bird's nests from the craft store and paper lanterns.

I cannot believe I missed the food and dessert table.  I was too busy eating!  Oh well!
We also had people write in this book.  We will continue to write in it every year.  I don't know if Nic will ever appreciate such a gift, but I know it will bring mom and dad back happy memories.  In the meantime, Nic smiles when I read it to her, and that's good enough for me.

Nic starts to laugh

It's official, we have giggles!

Gearing up for Easter....and learning some new skills

Nic has been working really hard at home in her therapy sessions and with mom.  We have mainly been working on range of motion for muscles, sitting up, maintaining focus on objects and the ominous tummy time.  Nic has been a trooper, while mom needs to work on letting Nic struggle.  Nic cries a lot during therapy now, and the sore muscles at the end of the day have been concerning.  No one in the world wants to watch their child struggle, and I view this as preparation (for lack of a better term) for the future.

Vision intervention (designed by mom and past coworkers) seems the most promising.  Nic recently had a functional vision assessment to give us an idea of what she is or is not using when it comes to vision.  The results are as follows:

VISUAL PROCESSING BEHAVIORS

The CVI Range developed by Dr. Christine Roman was used to assess visual processing issues due to the neurological diagnoses.    The scale ranges from 0-10 with 0 representing no visual responses and 10 representing resolution of lower level visual processing issues.  Higher level visual processing issues can persist if lower level issues are resolved.  Nicolette’s combined score was 3.25 placing her in Phase I which pertains to building more sustained looking behavior.  She is beginning to emerge into Phase II which starts to add function and meaning to vision.  The unstable status of the hydrocephalus could cause some regression on the scale but it generally expected that improvement on the scale will be realized due to the plasticity of the infant brain.  The visual processing behaviors broke down as follows:

·      Color:  Mother reported that red might be a favorite color.  Nicolette did best when targets consisted of 1-3 bright colors.  (Range 3-4; 0.25)

·      Movement:  Movement cues and reflective properties were instrumental in eliciting visual attention.  (Range 3-6; 0.375)

·      Latency:  A “slowness to visually respond” was present but slightly decreased as the session wore on.  (Range 3-4; 0.25)

·      Visual Fields:  The left field shortly left of midline was preferred.  Nicolette had great difficulties using all outer peripheral fields due to the head control issues and possible cataract interference.  Brain damage to specific fields could also be a possibility.  (Range 3-4; 0.25)

·      Complexity:  The environment needed to be controlled for all sensory input so that Nicolette would visually attend.  She preferred to use her sense of hearing over vision and became distracted when she heard another child crying in the hall.  Visual complexity of the target and background needed to be simplified.  (Range 3-4; 0.25)

·      Light Gazing:  Gazing toward lighted targets could be re-directed to non-lighted targets but responses were better to lighted targets.  (Range 3-4; 0.25)

·      Distance Viewing:  Purposeful visual responses to objects were limited to near space.  (Range 3-4; 0.25)

·      Visual Reflexive Responses:  The blink reflex was sluggish and the threat blink response could not be elicited.  (Range 3-4; 0.25) 

·      Visual Novelty:  Nicolette briefly viewed the new targets presented during the evaluation as long as they complied with complexity requirements.  (Range 3-4; 0.25)

·      Visual Motor:  Reaching attempts were not visually directed and look and touch occurred as separate events.  (Range 3-4; 0.25)

From this information, I then gathered baseline data on Nic's attending of color.  Nic is "looking" at the colors red, yellow, orange, and blue.  Blue needs a black contrast but she seems to love it.  Focusing times averaged are as follows:

• Red-Most preferred as expected with CVI-8.25 seconds
• Yellow-6 seconds
• Orange 5.25 Seconds
• Blue-3.5 Seconds

It is important to note that when testing I use a simple piece of paper or large toy that is a solid color and add a black background if needed.  I just added these photos because we also play, read books everyday, and well, she is SOOOOO cute!

These times give us a baseline to build off of to see if intervention is being successful.  I'm also currently awaiting the arrival of a copy of 

Roman-Lantzy, C. (2007). Cortical Visual Impairment: An approach to assessment and intervention. New York, NY, USA: AFB Press.

as Roman-Lantzy developed the scale of functional use of vision used in her assessment.  Her intervention in the meantime is trying to implement the following ideas (summarized by Pam Shanks, Primary Educator, Inclusion Specialist at Raintree Montessori School in Lawrence, KS):    

1.  Color and movement elicit looking:  Again what R-L would predict.  If she is not looking, moving the item a bit might help.  When she does fixate, allow her to look as long as she can.  Watch for her to "stop" looking.  You may have to keep the object moving to help her maintain gaze which frankly feels wrong at first.  You will want to stop making the object move when she looks.  Hopefully you will begin to see her length of looking grow and/or her ability to fixate, track etc improve. 

2.  Complexity: Invest in a black shirt or two and or a black apron.  That way you will always have an easy black background for presentation.  As you buy toys and items for her to use, think plain and one color (SO HARD TO FIND!!!!) and for now red (her favorite likely because it is easier for her see) or yellow.  See example below of ways to use red.  Red metallic curling ribbon bow would give movement (metallic reflectivity and actual movement) plus use red,  Add a black background to reduce complexity and a desk lamp (see below) for a spotlight and you have a great toy to elicit look and reach.

3. Spotlight vs. light table:  A spotlight (think adjustable desk lamp) can be very useful too.  Dimming the light and focusing the desk lamp on the visual target can elicit a look as well.  In fact, having a lamps as an alternative to overhead lighting in each room is a good idea.  Dim is good.  

Always put a hat or sunglasses when going outside. STILL WORKING ON THIS ONE as her helmet limits her ability to wear hats and sunglasses.

I will chart and post updates (hopefully) biweekly.

Currently, Nic is tolerating five minutes of tummy time per session at a rate of three sessions per day.  This is the hardest to work on, because she literally cries to the point of throwing up.  However, there are very few other ways to gear up her muscles to maintain head control.... And honestly, what a head she will have to control (HC 57 CM).  We take comfort in the fact that there is a notable difference in how much we (mom, dad, and therapists) have to "help" her with her head when sitting up to play with stuff.  This is incredibly hard for Nic.  There is generally what the next hour looks like after we work on this.

 But we try; we try everyday because that's our job as parents.  And we love her, and sometimes that means making our child push harder, work longer, and letting her cry a little.  Because, once she develops these skills, it will feel like winning the lottery, and all the sore muscles, all the crying and all the parental heartache will be worth it.

Dad is the best at sitting up time.  She loves playing games with him while working on trunk control.

 Tough work over here!

Also, I just wanted to note, that she confirm and monitor intervention with both of her PTs and her Pediatrician to make sure we are not cross in the line between hard work and hurting her.