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. 

Mom spends a birthday at Children's Mercy, and it was all worth it....

There are times as a parent of a child with special needs, that you avoid testing or assessments.  Maybe you think it isn't needed, maybe you think the doctor was wrong, or maybe-like me-you're just scared.  Maybe you can take multiple diagnoses like Hydro, CVI, PMG, etc. but maybe there is one that just brings you to your knees (literally).  For me, that dx was if Nic was totally blind.  In the NICU, we had a rather taunting doctor that repeatedly insisted we needed to “understand” that Nic was deaf-blind, that she brain couldn't possibly support the function of either sense.  This doctor followed us around so we "could fully understand the implications of Nicolette's conditions".  He even laughed at me when I read books to Nic in the NICU.

It wasn't that we wouldn't love Nic is she was deaf-blind.  It wasn't that we wouldn't try to support her if she was deaf-blind.  It was that I was terrified that I wouldn't have what it took to be a good mother a child with those impairments.  Could I learn sign language and other techniques fast enough?  Could I brush off the looks, the comments, the teasing fully enough?  Could I accept and table my own grief completely enough?

Deaf I could handle.  My SPED background told me the assessment timing of her ABR (just three days after surgery -WTH?!!!) just less than ideal and that maybe, just maybe enough to skew those severely impaired results.  We retested Nicolette's hearing in January.  It came back normal.  He husband nailed it when he said, "I never taught I'd see the day where 'normal' felt like winning the lottery".  He was right; that was exactly how it felt.

The second dx ate at me.  There were days Nic seemed to tract objects.  She looked at everyone's face when being held.  She didn't return smiles from people 3 feet away from her, but I couldn't see at that distance without my contacts.  Was she like me?  Tuesday, 4/7, I finally allowed Nic to be evaluated.... I finally "sucked it up" and realized that my fears shouldn't hinder our daughter's therapy.  If she was blind, then we needed to figure out what to do ASAP.  The world wasn't going to change for her; we have to prepare her for the world.

I'll never forget the fear inside me at her functional vision assessment.  It was like I cried inside myself for an entire hour.  And then it happened.... "I'm not one to talk about other medical professionals, but whoever told you she was blind should rework their assessment techniques."  My daughter does have CVR.  She does have a limited field of vision.  She will need help to learn to compensate for those impairments.  BUT SHE HAS VISION.

I'm learning this road is a long, long uphill route.  One with mislabeled signs, bad maps and scary detours, but now and again... just when you feel like totally losing it... you stumble across the best of views.  You learn how to become thankful for things you never realized were gifts.

I'm so CUTE!

Nic is enjoying her Pack and Play more and more each day.  She is developing a large following of stuffed toys!

She also is continuing to have the cutest outfits (IMO).

PT baby Steps

At three months, and more apparent at 4 months, I began to notice the developmental differences between Nic and other 3-4 month old kids.  Nic cannot left up her head at all.  How could I expect her to with a head larger than mine?  Nic was not laughing responsively like some babies were, etc.  I downloaded multiple developmental checklists like this one,
http://www.cdc.gov/ncbddd/actearly/pdf/checklists/checklists_4mo.pdf
and told myself to wait. I checkoff and date the items as she meets them.

I told myself not to get wrapped up in the details, but rather, I needed to focus on the progress.  AND Nic was making HUGE progress.  Then, as if the universe could feel the pain in my heart, hear the silent worry, it responded.   At 4 months, Nic turned into the incredible HULK.

On February 10, 2014 while holding up or supporting her trunk, Nicolette held up her head for 5 seconds.  This friday (2/21/14), Nicolette made strides in tracking colored objects, head turning (without her helmet, engaging her core muscles with head support, and holding her head in an upright position for 5-7 seconds.   My heart melted when Nic repeatedly held her head up with mild complaint for 3-5 seconds, approximately 5-7 times before telling us she was wiped out.  She is even tolerating baring weight on her feet!

As many of you now, the doctors at Children's Mercy really beat Nic's vision impairment into my head.  Nics optic nerve and occipital lobe were very underdeveloped as the inter cranial pressure was so great when it was time for them to form.  We were told she would be blind.  In the NICU, we could tell she reacted to light.  At 3 months, she began to stare at ceiling fans and started to track black and white toys.  Now she is tracking colored items!!!  There are many indicators that her vision is indeed impaired (e.i., needing to hold B/W objects very close to allow her to focus; Nic would stare at something, look away and then refocus again much like I see with children that have CVI; failure to return facial expressions).  However, yesterday Nic begin to responsively return smiles.  My heart melted.  I know Nic will accomplish her milestones at her pace on her own time.  I see her kicking, twisting, and crying at animal sounds that scare her (e.g., the lion on egg babies).  Everyday, I am filled with amazement at how far Nic is from the "unresponsive child" I was to bare. I feel so bless to know such a strong person.  I'd like to think she got some of that from me :)

Look at Nic finally "seeing" her punk rock princess rattle!

Valentine's DAY!

Nicolette celebrated her first Valentine's Day!  She even activated her core muscles during PT (with head support)!  We couldn't believe it; she just sat right up!  After PT, we spent the day making the best presents (see photo below).  Dad got the best print.  It took three people to paint her footprints, one to paint, one to press her foot and one to clean!  We then celebrated her 4 month birthday the following day!  What a weekend

Hydro Families and Austin

I'm back logging posts for a little while so I can get caught up.  I will try to reorder them once I finish so at least they can be read in chronological order.  In the meantime I also have to post some more current happenings.

Two weekends ago, Nic and I went down to Austin to meet many of the families that helped me through pregnancy.  This meet up was important because while no two hydro kiddos are the same, these children were similar to Nicolette in one way or another.  A little boy there and Nic shared close to the same head circumference (56cm I believe and Nic's at north was 58cm).  A little girl there also has a cyst like Nicolette although the placement is different (posterior as opposed to right smack in the middle).  Another child there had hydro to the same severity as Nic though she was birthed much sooner than Nic was (32 weeks as opposed to 37).  There was also another little girl that was as sweet as could be.  Nic and her share hydro and both have shunts.  :)

Friday, I drove to OKC to meet my hydro mom lunch buddy.  She travels to Wichita frequently, so it was nice to actually she her home and meet her precious five year old that I had hear and read so much about.  Saturday, we drive to Texas (6 hours).  Nic did really well and traveled in her car bed.  Saturday night was a meet and greet pizza night.  It was nice to actually talk to members of this group in person.  It was nice to have people hold Nic while I pumped, to eat dinner without worrying about Nic screaming and it getting cold.  Sunday was the best pancake breakfast I have ever had, a long talk with one of my favorite hydro mom, a trip to an inclusion, multi sensory playground (you can see in the picture below, Nic is not a huge fan of the great outdoors), and a dinner at a nice steakhouse.  Monday, we drove back to OKC, and by Tuesday, Nic and I were home... with a cold that she is still fighting!

While the events in Austin were laid back, the impact was profound.  As a parent, I cannot explain the overwhelming feeling of amazement and reassurance.  Each parent became my own hero in one way or another.  Each child was amazing and a miracle in his or her own way.  A nonverbal child signing concern when Nic was crying.  A child with a brain so abnormal, neurologist stop and stare for a large part of every appointment drinking everyone's chocolate milk because hey, they are HER cups.  When I first met the little boy, he smiled and said, "What's your name?"  I told him Nicole. "It's so lovely to meet you Nicole!  Can you help me find my chocolate milk?" OR when we were on our way to the restaurant Nic was super hungry.  I was pumping in the back when one child asked me what was wrong with the baby.  When I said she was hungry, the child looked at me straight in the face and said, "Well, you know what to do!"  Yes, yes Parker.  I do.

I guess what I'm getting at is through pregnancy I had so much fear.  I think anytime a healthcare provider tells you your child will never function like a typical child, you die a little inside.  OK, you die a LOT inside.  Parenting is met with total fear of the unknown; parenting a child with special needs is met with crippling fear.  How will the world except your child if they have a different world of their own?  It is terrifying.  If you're like me, you research everything.  You make the best decisions you can medically and emotionally.  You make a schedule and cross every PT's T and dot every medical release form's I.  You may even let your marriage fall by the wayside in looks to improving your child's future.  You prepare for the worst and hold desperately onto any kind of hope for the future.  You move day by day by day, and occasionally, you drive a 3 month old 9 hours to a different state to just sit by people who KNOW what you're going through, people who have been on the same road for much, much longer than you.  You look around and see that they are happy, they still have hope.  Then slowly, you wake up and realized that you have refilled your hope bucket, and that's enough to carry on, maybe even carry on with a smile.... Here's to you Nic.  Here's to you telling people it's so lovely to meet them or to you pulling off the baby's helmet because you what to see it up close, to you drinking everyone's chocolate milk while walking after having 11 brain bleeds, to you sitting in the car and deliver the justice of the must profound logical response.  Yes, the future does have light; I can see it as we walk through the tunnel.

Nic's 4th Surgery, A New NSG and a Fancy Helmet

As many of you know, we got to Duke and back. Nic is doing great. Unfortunately, we did need to have an additional shunt revision. This surgery turned out to be a blessing as it led us to Nic's new neurosurgeon. It was through this surgeon that we finally got Nic a protective/head shaping helmet. The best part about the helmet is that is gives her more protection, and thus, more people can hold her! We celebrate every step that gets us closer, and closer to being a "normal" baby. Nic is rocking at PT. She is meeting every milestone with the exception of motor skills involving head control. This leads us to the next part of her journey, the question is whether or not to get CVR for Nic. CVR is a Cranial Vault Reduction surgery that would decrease Nic's head size (remember it's bigger than her mom's head right now) and allow her to gain head control faster. Remember with head control comes rolling over, sitting up, standing and walking, skills Nic is not supposed to gain until she is 3-5 if ever. Dad and I are buried in research. Until the next update, enjoy these sweet pics!