Monday, September 29, 2014

We Can't EVEN - a.k.a. "A Feeble Thanks"

Y'all.  I can't even.

Sweet Snap from Eliz's bff.

Can't wait to wear our Hayes Bear shirts! XOXO MLE

(Yummy surprise on our doorstep from my sister's best friend.)


A church sister read about my disdain for washing/sorting socks... so she brought new ones.  HA!

Sweet Snap from one of my BFFs and her BBF (beautiful baby face).

Since our last post about Hayes' seizures, and even since we'd mentioned our concern about his 'spasms', we've been

overwhelmed

with an outpouring of love, prayer, encouragement, and generosity.

I'm not even posting pictures of the cards Hayes has received, the personal texts, Facebook messages posts, tags, and re-posts, retweets, phone calls, updates on shirt sales from our sister Elizabeth, faculty e-mails, and shout-outs we've been getting from ALL OVER.

I need better words.  I need better words to be able to say "Thank you... thank you, so much."  Those words seem feeble.

They seem feeble when I want to say, "You've transformed my recent "Please, please, please" prayers into "THANK YOU, Thank you!" prayers."

They seem feeble when I am bombarded (bombarded in the best way - like an over-abundance of cupcakes... or a battering of butterflies) on all fronts - Facebook, email, Instagram, Snapchat, text messaging, voicemail, real mail - by encouragement and assistance for Hayes.

Our family, friends, colleagues, employers, church family, friends of family, family of friends, friends of friends, (etc,) and STRANGERS we've never met have come to our aid by praying and showing support for Hayes.

I am so thankful and brought to a new mindfulness of the power of helping - and the many, many ways we're blessed in the current time to be able to help.

Y'all.  I am so guilty of not reaching out all the times when I have the power and ability to do so.  I think it takes a great courage to help someone.  It takes courage to say, "I love you and I'm thinking about you.  Whatever you need, I'm here."  THANK YOU for the courage you've shown in reaching out to us, for thinking and praying for us.

I am so thankful for social media.  What a wonderful instrument it can be in the arena of giving!  What an awareness it can raise for a family's needs!  What a way someone can feel able to help, when he or she may be too far to away make a meal, pick up a kid from daycare, or visit someone at their home.  I know this because I've watched social media help others, and now it's helping my family.

So, someone get on it.  Invent some new jargon that will echo the language, the hope and relief and happiness of my heart when I think of you all.  Then say that to yourselves, from us.

Pre-balded Hayes and semi-naked Knox.


And the Bear himself?  He's been in pretty good spirits since leaving the hospital.  We've ALMOST got all the glue used for the sensors off his head despite daily baths and various product attempts (no, I'm not joking - it is incredibly stuck and the amount of scrubbing it would take would cause a sore on his scalp, and I'm still not sure he's forgiven me from the haircut trauma I caused him last week).

His seizures seemed to stop while the ONFI was in his system, but beginning on Friday, he started having seizures again.  Today he probably had the most seizures I've ever witnessed him having.  Please keep him in your prayers.  We are going back to Cook's on Friday for a 30 min EEG, but we will have to wait to reconvene with our neurologist (also at Cook's) until next Wednesday, because they couldn't get those two appointments together on such a short notice.

AND YES, Knox is wearing Pull-Ups.  I really doooooooon't want to talk about it. (Potty training is hard. While you're on bended knee re: Hayes, would you mind sending up a quick one for K on this front?  95% kidding here,  That's 5% serious.)

We love and appreciate you all.  Good night.

Wednesday, September 24, 2014

My Son Hayes Has Epilepsy

My son Hayes has epilepsy.

That probably sounds more melodramatic than I really need it to be, but it's true.  In my last post, I mentioned that we'd noticed Hayes having what we'd previously called 'spasms' (which we now know are seizures) at the very beginning of September.  I contacted our neurologist, who ordered a 30 minute EEG for Hayes.

During the EEG, Hayes did not have a seizure, but we've since learned that even while his brain did not show seizure activity, it had patterns that indicated he was likely to have seizures.

The day following the EEG, I was able to capture one of his seizures on my iPhone and e-mail it to our neurologist, who immediately ordered a 24 hour EEG, which was finally scheduled a week and a day after the initial EEG.  I am so incredibly thankful that camera phones are a thing - there is no telling how many 30 minute EEGs we would have had to endure before one caught a seizure.

The night before the 24 hour EEG, I decided to minimize the pain Hayes would experience while attaching/detaching the EEG sensors by cutting his hair. Things escalated quickly - Hayes was not taking kindly to the hair cut, and fearing that he'd inadvertently make me stab him with the scissors, I reverted to the clippers.  And now, he is bald.

Yesterday we arrived at Cook Children's Medical Center at 10:00 am (or a little after, since I had to turn, turn, turn, up to the very highest level of the parking garage - that hospital holds A LOT of people) to begin the 24 hour EEG.  I was so relieved that we weren't having to sleep-deprive Hayes for this observation, like we did for the last EEG.

Hayes had a seizure almost immediately after he was hooked up to the monitor, around the peak time frame that I'd mentioned noticing/making note of his seizures.  (I've noticed him generally having them in clusters of about 10 minutes or less apart a few hours after waking and before lunch time, and then in the later afternoon and early evening.  But, he will occasionally have them in sporadic isolation or clusters at any time throughout the day.)


Hayes slept a lot during the day ... (more about THAT later...) but he did have a few more seizures that we were able to mark.  Each time Hayes would have 'an event', I would push a red button and the people watching via the video camera in our room (he was humanly-monitored as well for the entire duration) would mark it on the computer so the physical "event" would correspond with the brain activity.

Around 3:00 / 3:30 (while Hayes was still sleeping), the epilepsy-neurologist specialist came in to let me know that the seizures that Hayes had already had were classified as myotonic clonic.  Myotonic clonic seizures can be damaging to the brain, and to stop them he recommended we begin administering the anti-epileptic drug ONFI.  (*30 minutes pass as I ask this doctor probably every question I can think of about the brain, the seizures, and this drug*)  The seizures are spasm-like jerks of the muscles, which you can see in the video at the very end of the post.  The doctor left, and Hayes had a very late lunch.



Koby arrived just before 7 pm and we took turns going to grab dinner in the cafeteria below, then bringing the food up to the room to eat.  Hayes dozed after having another bottle and then had dinner around 8 pm.

A poor, unsuspecting pharmacist came in to brief me on ONFI and stayed 20 minutes longer than he'd probably expected while I asked him to describe the reactions of every human who has ever taken this drug.  No, okay.  But almost.  

Around 9 pm, Hayes' nurse came in to administer his first dosage of ONFI.  

Let me quickly tell you that anti-epileptic drugs generally work because they depress, or slow down, the nervous system.

Except for Hayes.  

Side note - Hayes can get hyper sometimes.  In fact, almost immediately after he was hooked up to the EEG monitor, I took him out of bed in order to give him a snack.  He began kicking and thrashing about in his excitement of THE FOOD THE FOOD THE FOOD.  Unfortunately, the people watching the video surveillance have not had the pleasure of seeing my son Hayes eat before, so I was a little confused when I heard, "We're coming, Mom (everyone called me "Mom") and then a nurse busted through my door saying "OK! Something happened?"

And I said... "Yes... he saw the apple sauce."

This is Hayes' "I'm eating food" dance, complete with new beat-boxing skill and kick-finish.


video


Apparently, my son expecting food looks like a grand mal (tonic clonic) seizure.

Back to the story - enough about that.  HAYES ON ONFI = that hyper kid you remember from elementary school who should have been medicated but never was.  Except on crack.

Oh, and remember how I'd let Hayes sleep all day?

Hayes beat-boxed and clapped and yelled (loudly) until about 12:30 am, when he remembered that he hated all the wires on his head, at which point he began crying (loudly) for another 30 minutes.  We finally gave him some Tylenol, and he was able to rest.

At 6 am, Hayes had some blood drawn for a lab test that his endocrinologist orders every 6 months.  Unfortunately, this didn't go well and ended with him being stuck in both arms before we could get the blood he needed.

Hayes dozed on and off before breakfast.  Koby and I again took turns in getting breakfast.  We hoped we'd be leaving around the 24 hour mark of Hayes' arrival (10 or 11 a.m.) , but we began to suspect from the nurses' indirect answers that it may not work out that way.  (Side note - all the nurses and doctors and family liaisons and social workers and chaplains and social workers again and then family liaisons who came in our room again were WONDERFUL, as are most of the employees we interact with at Cook's.) 

Finally, the neurologist came in between 2 and 3 pm, after we'd learned he'd wanted to confer with his colleagues regarding Hayes' video EEG.  He let us know that they'd identified an additional type of seizure, this one called complex-partial.  These seizures are harder to recognize, as they don't generally involve a convulsion of the muscles.  When Hayes has them, he 'blanks out' and stares into space, and they last for about 7-10 seconds.  This concerned Koby and I, because Hayes has been doing that almost his whole life, which I've brought up with our neurologist before.  We'd attributed it to sensory-processing overload, almost like a coping mechanism.  

Because of this additional type of seizure, our epilepsy-specialist neuro wanted to hold off on the ONFI until we tried a 5 week course of oral steroids.  Steroids cannot be administered for long periods of time because of the side effects, so he wanted to try this first and not in conjunction with the ONFI, so as not to confuse which medication was effective or ineffective.  

So.... no ONFI.  Steroids.  (Enter 1,000 questions.  Click here to see one of the sites I visited after thinking, why steroids?)  Steroids can be immune system suppressors, so Hayes will be avoiding crowds/daycare for the next five weeks while we begin a steroid regimen and then wean him off of the medication.

And then we stayed at the hospital for another three hours because of complications with a urine lab (it leaked) and my prescription insurance (I haven't got the card yet) ...

And then we were driving home and it was wonderful.

Hayes convinced a sympathetic nurse that he didn't need the awful gauze on his head, and he promised to stop tapping and picking at it.
 Hayes having seizures isn't wholly alarming or shocking to us - his potential risk for having seizures is one reason we've been seeing a neurologist for over a year.  In essence, this will just be an adjustment as we begin medications and learn to live with seizures (hopefully not for very long!).  We know that his seizures are just symptoms of the underlying problem that we have yet to identify.

We are desperate to end these seizures because we want Hayes to reach his full potential, and these episodes inhibit his brain from progressing.  We want him to keep moving forward and learning and achieving until he is doing all that he was and is able to do.  We know that his full potential looks different than most others', but we are so grateful for the things he is able to do, and dread him losing those abilities.

Koby and I want to thank our families, coworkers and employers, caregivers, therapists, and friends who have been a constant support in this new development with Hayes.  We have had teachers cover our classes at almost a moment's notice, friends pick up Knox from daycare and offer to bring food to the hospital, and family who are willing to continually pray, encourage and sacrifice their time, resources, and energy to ensure that Hayes is offered the best-possible healing environment.  Hayes' and Knox's school caregivers are wonderful and flexible, and Hayes' therapy and intervention team is second to none.  THANK YOU ALL for your prayers and encouragement.  We feel very loved and lifted up.

video

For those interested, this is a video of Hayes during one of his myotonic clonic seizures.  The seizure happens within the last seconds of the video.  You can see that he is upset or disoriented after the event, which is a normal occurrence after the seizures.

Wednesday, September 17, 2014

Hayes' Spasms



The purpose of my Paints and Pistols blog (which probably needs to be renamed) is to inform interested parties about my family: keeping friends and family updated on our goings-on, informing curious parents and family members about various medical terms in the plainest of speech, and hopefully making readers laugh 6% of the time.

I started an informational series on Optic Nerve Hypoplasia, which is a brain condition affecting the eyesight (among other things), and my younger son Hayes has it.  But I've neglected to post since the very beginning of the summer, and I have realized over the past months that focusing solely on ONH on the blog is misleading to (most) people who don't know Hayes and what's going on.

Hayes is dealing with quite a bit more than ONH.  At the very end of summer / beginning of the school year, Hayes had his second MRI, which showed 'white matter loss' in his brain.  To put it so simply that it may be mostly incorrect, the 'grey matter' in your brain is where information is stored, and the 'white matter' is where information is transmitted, or carried and connected, throughout the brain so you can use it.  Hayes' 'white matter' did not show up in the amount that is normal for an almost-two-year-old brain.  We don't yet know if this is because these parts of his brain are completely without function, or because of something called 'delayed myelination', which essentially just means that his brain hasn't made the connections yet that would make that part of his MRI picture show up the way it should.  For this reason, he'll have another MRI in a year, to see if his brain continues to develop or stagnates.

We know Hayes is globally developmentally delayed, meaning that he reaches his 'milestones' across all categories (mobility, social, cognitive, etc.) more slowly than most other children.  Which would obviously explain why certain connections are not showing up on an MRI picture.  What we don't know is WHY (he has no official diagnosis that explains his disabilities and for this reason we are in the sloooooow process of genetic testing, which has not given us any knowledge thus far except that we cannot depend on our local labs to get blood work to the places the blood work needs to go) or WHAT WILL HAPPEN NEXT.  (But who really does, anyway,..)

About a week ago, we began to notice Hayes having some spasms, and they seem to have increased in frequency since.  These instantaneous spasms last for a mere second, and usually involve Hayes falling forwards or to the side, dropping his head and/or hunching his shoulders/raising or extending his arms.  He seems confused afterwards.  I called our neurologist (brain specialist) the first day I noticed him doing this more than once (he may have done it in isolation the previous week/weeks, but I'd assumed he'd startled himself), and we were able to get in for an EEG on Tuesday (yesterday).  An electroencephalographic (EEG) reading notes the electric impulses the brain makes during different activities.  We hoped to catch some of Hayes' brain waves during spasmic activity, but he did not have an outright spasm during the 30-45 minutes of the EEG, most of which recorded his brain during sleep.  (More aptly named EEN: extremely expensive nap.  <<< This is that 6% I was talking about.)

Though we're still waiting on the official EEG results, I was able to video Hayes having one of his spasms last night and emailed it to our neurologist this morning.  Based on the video, our neuro ordered a 24 hour surveillance study of Hayes, to better determine these 'concerning' episodes (his words), despite anything our EEG results show/do not show when we get them over the next few days.  This appointment is yet to be scheduled (I should hear tomorrow, or so they tell me as I keep calling).

There are a few conditions/types of seizures we're discussing that I'll expand on in more detail once I know for sure what I should put out for every one to read.  We're concerned, obviously because some seizures can slow and regress development, rendering the victim with brain damage.  In Hayes' case, we are especially wary of this and ready to begin measures to control seizures (if they are, in fact, seizures) as his brain is already working around other developmental issues/damage.

We (Koby, Knox, Hayes and I) covet your prayers during this time of testing and waiting - prayers for answers, peace, and effective treatment for Hayes.  Thank you for your encouragement, support, and concern for our sweet son.

Thursday, June 26, 2014

In Summer

You know those mom characters who are always on TV like...

"I know from the outside, I look like I have it all together, but I'm really stressed out on the inside, blabbedy bla bla bla..."?



(I know you're confused, but that's not me.  That's Claire Dunphy from ABC's Modern Family.  You may have forgotten what I look like since it's been approximately nine years since I last blogged.  A lot happens in nine years.)

I feel like I might be the opposite of ^^^ that.  Usually, my life and my general demeanor gives off the impression of Hot Disorganized Mess, but I feel relatively calm.  Or also I feel that way inside, too.  Either way, I'm one of those special ones who doesn't have it all together, and it also appears that way.  So what I'm getting at is, where is my show?

Anyway, I'll catch you up.

We wrapped up the end of the school year with me being asked to move up and teach 7th grade English next year and to take over the middle school Gifted and Talented program.  I said yes, which means I'll be moving up with the students I had this year and teach a tested subject for the first time in my career.  Should be interesting.

Koby was super happy to end the year because post-basketball season, he'd been getting the boys ready and taking them to school each morning sans help from Mommy.  Interesting outfits and hairdo's, y'all.  It was a glorious experiment and I have already been informed that we will not be engaging in the experiment next year.  But in all seriousness, I was really happy for the break.  Thanks husband!



Knox was pleasantly surprised to find that he'd be staying home, for like, a week.  After that he began to say really dramatic things such as "I NEVER get to see my friends anymore," and hopeful things: "Are we going to school today?"  He took swim lessons from a friend in Graham a few weeks ago, and I was really happy for him to have that experience.



Hayes is doing really well and has been making great progress in the past few months - he's discovered his feet and is overall more engaged with his surroundings.  It's been exciting to watch and enjoy.  We've kept up with our therapies, which give me motivation to vacuum, as they take place at the house over the summers. (Six occupational therapy sessions, two vision instruction sessions, and one speech therapy session each month.  He's a busy boy.)

We have been traveling (A LOT) and summer schooling and GT training and avoiding cleaning our house at all possible costs.  We've been to LOTS of doctor's appointments, bought a new car, fished, watched Frozen 64 times, house sat for Koby's parents, visited mine, and made the house a bit messier (still not sure how, since it feels like we're never here?).




Coming up: possible family camp for families with blind children in August (we're on the waiting list), a second MRI for Hayes (August 14, prayers appreciated as he will be fully sedated again), more doctor's appointments, Knox's third birthday, two-a-days, coaching camps, coaching school, a weekend in Junction, Texas, Koby's and my fourth anniversary, and a miraculous cleaning of the house.

I've been struggling lately with the natural self-absorption that comes with having young kids.  Getting away from the god of 'Busy'.  I do want to bless others and teach my kids to be looking for opportunities to help in all occasions, but feel like so much of my time (effort, money, etc.) is taken up with grilled cheese sandwiches, laundrylaundrylaundry, commutes to doctor's offices, and general LIFE.  I really wanted to write some amazing post on it, but my friend Sarah beat me to it and said everything I'd been thinking, and is actually putting it into action.  If you feel like you simply can't break the cycle of "US US US" or "ME ME ME", go check it out.  (And I mean it - simply.  She offers really simple ideas.)  No, go check it out anyway.

Sunday, April 13, 2014

What IS Optic Nerve Hypoplasia? - Everything I Know Part 2 -

Okay, we're back.  I hope from my last post you were able to come away with a general overview of what optic nerve hypoplasia is.  If you didn't catch that post, or if you've stumbled upon my blog because your child was just diagnosed with optic nerve hypoplasia, I'd recommend beginning here for information, or here for the beginning of our own story with ONH : )

In January I attended a conference put on by the Texas School for the Blind and Visually Impaired (TBSVI) specifically for parents of children with ONH.  At this particular meeting, we were able to hear information from an ophthalmologist who had become something of a specialist regarding ONH.  I loved what she said at the very beginning - ONH is a brain problem.  It's not an eye problem.  That's why vision isn't (always) the only thing affected by ONH.

The optic nerves exist in a part of the brain called the 'midline'.  Because kids with ONH obviously have malformations in this part of the brain, one of the first things that happens (or SHOULD, MUST, MAKE IT HAPPEN) after an ONH diagnosis is a referral to an endocrinologist and an order for an MRI of the brain.

Below are two pictures of different views of the brain and its regions/parts/whatever you call brain stuff.

The first is a side view - the midbrain that is usually checked for additional abnormalities after the diagnosis of ONH includes the corpus callosum, the hypothalamus, and the pituitary gland.  (Image courtesy of a Google search, which yielded this picture in a foreign language.  Good luck with the link.)


This picture (below) shows a view of the brain from above.  You can see how the optic nerves cross just in front of the pituitary gland in the middle of the brain.  (Image courtesy of some lecture notes from a community college.  Knock yourself out.)


Okay, so what does this all mean?  Why are these parts of the brain important?

First and foremost, let's discuss the pituitary gland, aka the reason you'll be referred to an endocrinologist (doctor specializing in glands/hormones).  The pituitary gland is responsible for secreting the hormones that keep the body functioning normally.  Because hormone abnormalities can have drastic consequences (loss of consciousness, mental retardation, even death), it is imperative that a child with an ONH diagnosis be monitored for pituitary function.  And because the hormone levels can fluctuate at any time or stage in life, people with ONH should continue to be monitored with regularity even if everything is functioning normally.  Sources vary, but it is reported that 75-80% of people with ONH have some degree of pituitary dysfunction.

The hormones secreted from the pituitary gland are responsible for the following things:


  • growth, temperature regulation, sex organs, thyroid gland function, metabolism, water balance/regulation in the body (related to kidney function as well), blood pressure, and pain relief 


Your endocrinologist will regularly monitor your child's hormone levels by ordering blood tests and, at each appointment, check to make sure she is growing normally.  This specialist will also review the results of your MRI.

Hayes' hormone tests have come back in the normal range after each screen.  He has always 'thrived', which is to say, grown and eaten normally.  He is tested every six months and will be until he is 2 (or 3, can't remember).  At that point, if he is still maintaining 'regularity' as far as his hormone levels, he will only be checked once a year.  Of course, if there are any changes in behavior or activity, we will contact his endocrinologist to run tests to make sure something hasn't gone awry, because hormone problems can manifest themselves at any time.  (For more information about the pituitary gland, talk to your endocrinologist and click here.)  And, for the record, I love our endocrinologist.  He is hilarious and looks like Dennis Hoffman, and he thinks I look like Brittany Murphy.  I was not as thrilled and am still confused about that.)


This is Hayes and me at his endocrinology appointment on my birthday in 2013.  As you can see, he's chunky thriving, and I look nothing like Ms. Murphy.  No offense to her, and may she rest in peace.)

Let's talk about the MRI.  An MRI is an image done by magnetic resonance imaging.  I have basically no idea how this works.  Okay, I have some idea, but I feel more comfortable explaining why a person with ONH is ordered an MRI than how exactly the MRI is done.  (For much, much more re: the MRI, please talk to your endocrinologist/neurologist/ophthalmologist and click here.)  The MRI for ONH will be focused on the brain, and will be specifically taken for review of the midline.  

We had a few issues as we went to have the MRI done in the spring of 2013.  Hayes was getting over RSV and had recurring colds, which led to us postponing the scan once before we finally got to do it.  I was a nervous wreck.  Because Hayes was only six months old when he had his MRI done, he had to be put under anesthesia in order to ensure his absolute stillness.  He also had to fast (which, at that time, meant no bottles) for a period of time before the MRI was done.  The whole thing took a whole morning - arriving at the hospital at its earliest opening (some tortuous time like 6 or 7 am, and my mom and I drove in from Jacksboro for the appointment), and after he went in around 8 or 9 I didn't see him until after lunch.  I tweeted a lot about it.  Here's a link to a tweet/picture of Hayes in his hospital gown, 10 hours since last bottle and pre-anesthesia.  You can find me on Twitter by my handle @christieandrews .)

What else is the MRI looking at?  We already talked about the pituitary gland.  So next, the corpus callosum.  The corpus callosum is a group of fibers that connect the left and right sides of the brain.  These fibers allow the sides to 'talk' to each other.  Here's something completely mind-baffling about the corpus callosum: it can be completely missing and you might never know.  Some people with absent corpus callosums go on to college: some with partial loss of the same section of the brain have significant cognitive and motor impairments.  When the corpus callosum is thin, malformed, partially or completely missing, it is called agenesis of the corpus callosum.  (For more information about the corpus callosum, talk to your neurologist and visit this site.  A wonderful organization exists called the National Organization for Disorders of the Corpus Callosum, but unfortunately its website is temporarily down.)

Attached to the corpus callosum is the septum pellucidum, which is a thin membrane that runs to another part of the brain.  A condition often associated with ONH, called SOD (Septo-optic Dysplasia) involves diminished optic nerves, pituitary gland dysfunction (hormone problems), and a missing septum pellucidum.  For more information about SOD, talk to your ophthalmologist, endocrinologist, and/or neurologist and click here

I took the call regarding Hayes' MRI results on April 24, 2013.  As I'm writing this, that's only 11 days less than a year ago.  Hayes HAS a corpus callosum, but because he was six months old at the time of his MRI, his brain was six-months-old-tiny, as was his corpus callosum.  It's hard to tell, at that size, if a corpus callosum is malformed.  Hayes has not been diagnosed with SOD, as he doesn't seem to be 'missing' any brain parts and he has yet to have any endocrine issues.  Since his MRI, I've been wondering if his corpus callosum could be linked to some of the motor problems/delays he has, which you'll learn about later.  If your child has been diagnosed with SOD (or ONH), I would highly, highly recommend joining the Facebook group called 'Parents of Children with Optic Nerve Hypoplasia (ONH) / and Septo-Optic Dysplasia (SOD)'.)

Alright.  So.  We know that an ophthalmologist will refer a patient with ONH to an endocrinologist, and hopefully order an MRI (and it's not just to spend money; we understand why.).  Who do we discuss the MRI results with (aside from the endocrinologist... and the ophthalmologist.... and your family doctor/general practitioner/pediatrician...)?  We go to the neurologist.  A neurologist is doctor specializing in disorders of the nervous system, aka the brain.  And, obviously, ONH is a disorder of the brain.  The neurologist will (should) review the MRI results with you in detail.  She should show parents the MRI itself, discuss the findings in each targeted area, and explain what those findings mean.  A neurologist will be the doctor contacted in case of seizure activity, since that can be and is associated with ONH.  If the MRI results are inconclusive for any reason, the neurologist will refer you to other specialists.  (We'll discuss that later.)

I like our neurologist as a human and as a professional.  I really just haven't learned anything from our appointments with him.  He showed us Hayes' MRI results (pretty cool) and is willing to answer my ridiculous please-predict-the-future questions, but because Hayes has some other things going on that aren't explained by his MRI, he's referred us to other doctors.  We check in with our neurologist every six months to discuss any changes in behavior, monitor for signs of seizure activity, and keep track of his development.  The neurologist was the one who FINALLY said 'hypotonia' out loud for me, and is the one who is most concerned about his global developmental delays (which means delays in cognitive, speech, and gross/fine motor skills).

And from this point?  Rinse, lather, repeat.  And adjust as your preferred brand of shampoo is renamed, discontinued, or new and improved.  Because this is the medical community.

So, a recapitulation of post-ONH diagnosis and the 'D-team' assembly. (D is for Doctor, not performance.*  Hopefully.)

ONH diagnois (most likely via the ophthalmologistthen


  • Endocrinologist
    • hormone tests
  • Neurologist
    • MRI
Everything I Know Series
What IS Optic Nerve Hypoplasia - Part 2 (you are here)

  • LEGEND
  • Medical information / New Terms
  • Personal Andrews information
  • Medical specialists
  • Therapy / Government Programs
  • Really important information
  • and links I think you should visit always look like this

If all this helps just one person, I will feel incredibly accomplished.  It's a lot of typing if it doesn't.  :-)  I'm going to condition you to expect a really cute Hayes picture at the end of each installment, so here:

What?  Stop it, I know.  Hayes and Koby catching a snooze after a long weekend.  Hayes is smiling in his sleep.
* Remember, you can fire anybody who isn't up to snuff.  You can always question, challenge, and ask for clarification.  Above all, if you aren't satisfied, you can find another doctor (therapist/case manager/etc.)

Tuesday, April 8, 2014

What IS Optic Nerve Hypoplasia? - Everything I Know Part 1 -

It's come to my attention that everyone who reads the blog knows something's up with Hayes.  A little over half know that he has a neurological condition (a congenital birth defect) called optic nerve hypoplasia (ONH).  Less know, however, what that really means and entails.  And a few know there's some other stuff going on too; things that no one can really describe well or explain.

I think that sometimes even really, really close loved ones can feel uncomfortable when asking parents whose children have atypical development to explain, in detail, what's going on and why.  And so, they don't*.

I also know that I have read ... a lot... of information (printed and online) about optic nerve hypoplasia; I still Google it sometimes to see if anything new comes up.  I know how it feels to be a frightened mother looking to educate myself on something I'd never heard of until it affected my baby.

SO I want to do an exhaustive post (series) that covers everything that we know about ONH because of Hayes.  Please keep in mind that I am neither a doctor nor a pediatric therapist, and I am describing our family's experiences and decisions regarding this condition.  I'll post medical information, signs, and links I consider to be useful resources.

Optic nerve hypoplasia is a congenital (before birth) neurological (brain) condition resulting in varying degrees of vision loss and can be associated with other disorders and conditions.  Hypoplasia means 'under-developed', and so the name of the condition itself basically describes the fact that the optic nerves were malformed as the baby's brain developed during pregnancy.  The optic nerves are the nerves that connect the eyes to the brain.  There are no known causes of ONH, but studies have linked the usage of drugs, young maternal age, primaparity (first pregnancy), and low maternal weight gain to the condition.  It is not known to be a hereditary condition and is not currently linked specifically to any chromosomal malformations.

When a person has optic nerve hypoplasia, it can affect one (unilateral) or both (bilateral) eyes, and the development can be moderately to severely under-developed.  For example, one person with ONH may have optic nerves that are only slightly smaller than the typically developed person's, while another may have optic nerves that are almost completely diminished.  Obviously, this means that even with the same medical condition, vision loss can range from near to normal acuity (what we call "20/20") to no light perception, meaning that the brain does not perceive the image of light through the eyes.

Every ophthalmology appointment/MRI result has indicated to us that Hayes's optic nerves, while smaller than average, are not markedly underdeveloped.  Unfortunately, as he is still unable to talk, we are not sure of just how much/well he is able to see.

It is important to remember that even with a bilateral ONH diagnosis that the brain will adapt to the under-developed optic nerves.  And so while the optic nerves will not themselves repair or grow stronger, the brain can learn to read their signals better over time, often until the ages of three to five.

This is also why it is important to monitor the health and function of the eyes.  ONH is sometimes associated with strabismus (eye crossing) and nystagmus (eye shaking).  In the case of strabismus, if the eyes are crossed for a long period of time, the brain will eventually 'shut off' the signals it receives from one of the eyes to cancel the 'double vision' effect.  (This is called amblyopia.  Read more about it here.)  Treatment can be consulted with your ophthalmologist and generally consists of patching either eye or undergoing strabismus corrective surgery. (For more information on strabismus, talk to your child's ophthalmologist and click here.)

It was because of strabismus that we were initially referred to a pediatric ophthalmologist by our family physician after Hayes's four month check up, and in turn it was the ophthalmologist who first diagnosed Hayes with optic nerve hypoplasia.  Hayes had the strabismus corrective surgery in July.  His eyes have healed nicely and do not cross as frequently.  Because both of his eyes have always seemed to cross equally, rather than one or the other, our doctor has never recommended patching, and for this reason I am less comfortable giving extensive information on patching.  (For more information on patching as an aid for eye alignment and acuity, talk to your child's ophthalmologist and click here.)

Nystagmus often occurs in people with ONH, and it means that the eyes move quickly, or 'bounce' in different motions, like up and down or side to side.  Interestingly, people with ONH can find their 'null point, which is an angle at which they can look at things without having their eyes move constantly.  (For more information on nystagmus, talk to your child's ophthalmologist and click here.)

We have been on the watch to see if Hayes seems to prefer looking at objects or people from certain angles, which may indicate that he has found a 'null point'.  There is actually a surgical procedure that can 'move' a person's null point.  For example, it would be much more convenient if the null point could be found when the eyes were looking straight forward, instead of looking at things obliquely from a wide angle.  So far, we have not noticed Hayes preferring any such positioning.  

We have noticed him 'nodding' quite a bit, and at this last appointment, the ophthalmologist referenced a condition called spasmus nutans that is associated with nystagmus, although not necessarily with ONH.  Spasmus nutans is characterized by nystagmus and head movements, such as nodding.  It generally manifests during infancy and early childhood, but generally resolves itself by ages 3-4.  Because the signs can also be misread as spasmus nutans when they can actually be signs of problems such as brain tumors, doctors will often order MRI scans to be done.  Since Hayes has already had this done with no concerns coming up, our doctor felt comfortable ruling that out.  (For more information about spasmus nutans, talk to your child's ophthalmologist and click here.)


Because no one likes to scroll forever, this wraps up the first part of what I hope will be an informative, frequently updated series about optic nerve hypoplasia and hypotonia (in broad scope) and our journey with Hayes (in specific detail).  Stay tuned for: associated conditions, posts on early childhood intervention and how to get it, hypotonia, global developmental delay, genetics, and most likely more.


Everything I Know Series
What IS Optic Nerve Hypoplasia - Part 1 (you are here)

  • LEGEND
  • Medical information / New Terms
  • Personal Andrews information
  • Medical specialists
  • Therapy / Government Programs
  • Really important information
  • and links I think you should visit always look like this

But here's your reward for making it this far.  Hayes says, "I bet you thought she'd never stop talking!"


*It's okay to ask.  : )

Saturday, March 1, 2014

Prophecies, Progress, and One Identity-Stealing Transformer

I'd love to take you back to this moment, as I wrote this when Hayes was just ten days old:

A second child is a heart-breaking miracle, a treasure mindful of time.  He is precious because his parents have learned the meaning of cherish.

Okay, so maybe the postpartum hormones were a-swirlin' that day; I know it's a tad on the dramatic side. But I've been revisiting that thought over and over again.  It's almost like my own personal memory verse that became something of a self-fulfilling prophecy for Hayes and me.

I would lie if I told you it isn't hard when Koby and I attend Hayes's pediatric check-ups; we sit there and say "No" for about five minutes straight as the nurse keeps asking all the questions she's bound to ask about his development.  "Is he walking?" "Is he crawling?" "Is he pulling up on furniture?" "Is he babbling?" "Does he play hide and seek?" "Will he look for missing objects?" .... Please, please stop, we think.

I would lie if I told you it isn't hard when we see kids Hayes' age running and laughing and talking.  Because it is.  It's not a resentful, pitiful feeling.  But it's a hard one.

BUT.  Our little treasure mindful of time keeps reminding us that HE progresses on his own time, and it transforms the ordinarily mundane into the explosively joyful.

Yesterday, Hayes grabbed my face and looked into my eyes.  He just ran his slobbery, pudgy hand all over my face, looking at me and studying me in a completely new way.  

For any other mother, if her 16 month old did that, it would probably be answered with - Look little man, here are your 10,000 toys.  Could you please pinch and play with them instead of Mommy's cheeks and hair?  I know and say this because that is exactly what I would have felt when Knox was ALWAYS UP IN MY GRILL about a year or so ago.

But when Hayes did it - purposefully reached for and held my face - it made my heart race and flutter.  CHERISHED moment.

video

Here's a video of Hayes in his Little Room.  The Little Room is an Active Learning therapy tool we've been able to use thanks to our wonderful VI teacher.  He's laying on a Resonance Board; the Little Room is the torture-chamber apparatus that appears to be encasing him.  This was his first time in the Little Room, and not coincidentally, this is the most he'd ever interacted visually-physically with ANYTHING.  (I've since moved some of the objects around - I didn't like how close those spiky dish scrubbers were to his face/eyes, and I let some of the higher-hanging objects down a bit.  It is recommended to establish places for the objects -and then not move them- for memory encouragement.  And obviously, children should be monitored the entire time while they're in the LR.  Don't mind the other, non-monitored child destroying the living room in the background.)  For more information on Little Rooms, Resonance Boards, and Active Learning, check out these links:

This Link
or This Pinterest Board
or This Link


Heartfelt outpouring over - I have to go attend to Knox, who is refusing to believe me that the toy he is holding is. in. fact. a Transformer, and not a Yellow Batman.  



** Update: I later apologized and said, "Okay, you're right.  It is Batman.  How silly of me."  To which Knox replied, "Good job, Mommy."  These guys teach me lessons all the time.**


Saturday, January 4, 2014

Keeping the Dementors at Bay

Where did the end of 2013 find you?

Or, let's start this way.  Have you ever had one of those moments where you're almost able to step outside of yourself and take a good, long look at the human being that you are?  Or a short, but still very alarming, look?

That moment came for me at the end of 2013, as I found myself crying on the way out of a Walmart Supercenter in Mineral Wells, Texas.

Backstory: I had meticulously planned a NYE trip for Koby and myself that was going to be stunning, stellar, scrumptious, except for the host of signs that we should cancel the excursion ... and then, to top it all, forty five minutes after leaving the house, I remembered that I forgot my dress.  The dress.

Further Backstory: Never considering myself exceptionally 'materialistic' or 'vain', I  have always figured I might be exempt from these annoying bad habits as I am so conveniently without 'style' and the womanly ability to use makeup or style my hair well.

Cut to: the moment when I am crying in the third store my poor husband and I (well, just I) have raided in a blind and very real panic; I am LITERALLY WEEPING WET TEARS FROM MY EYES because all the dresses my hands have touched are apparently 'too ugly' to put on my body.  I experience a very real longing for the designer dress, worn only twice because it isn't church-appropriate and where else do I go?, hanging in my closet.

I told myself I wasn't a snob, not really.  I tried to explain to Koby the urgency a mother of two feels about looking hot JUST *ONE* NIGHT A YEAR, for crying out loud.  I tried to tell myself I was just anxious about spending more money on something I didn't really need or love, and thus I was ACTUALLY being financially responsible...

And then I awarded myself Miss First-World-Problems of the Universe, laughed at how ridiculous I was being, found a perfectly acceptable dress, and went on to have a stunning, stellar, scrumptious evening with the Best Husband of the Universe, who still can't tell the difference between the grey dress I left at home and the black one I wore to dinner.



As a collective unit, 2013 was like a bottle of wine; it most certainly improved with age.  Early 2013 was extremely bitter and hard for our family; Knox and Hayes were sick A LOT, I was anxious because I thought something might be wrong with Hayes, then Koby was anxious because I told him that, then our world was rocked when Hayes was diagnosed with ONH, and then he was sick some more and we postponed an important MRI, then I wasn't sure if I'd have a job the following school year...

And then I did, and it was better.

And then we moved and it was great.

And then Hayes started ECI with his wonderful therapists.  And then he had a successful eye surgery.  And that was awesome.  Koby and I celebrated three married years and Knox turned two.  Boom, boom, pow.

Finally, Knox stopped being sick all the time (knock on wood), and that may have been a miracle.

And then Koby had a great football season, Hayes turned one, and Koby grew a great beard.  And we topped the year off with lots of time with family and a fabulous New Year's Eve. (Thanks again to Lovie and Popi for babysitting!)



Personally, 2013 has been an interesting combination of self-doubt, insecurity, and confidence.  As I grow into the role of 'mother to a child with special needs' (Koby HATES it when I say that), I experience the gamut of emotions to be expected with its unique responsibility.  For any mother to a child with congenital birth defects, the guilt and fear can strike at any time, even after months without it: for me, it's usually when Koby is gone.  I find myself frantically wondering again - did I do something wrong?  I am a slave to Google - was it the off-brand prenatal vitamins that I took?  Was it the glass of wine I had four days before I found out I was pregnant?  Was it the doctor-approved exercise?  WHAT WAS IT???

This year, worry has been my dementor*.  Worry sucked the meat off my bones and the joy out of more than a few days this year.  Worry led me to guilt like that; and I also found myself worrying, for almost the first time in my life, what others thought about me. (In fact, I had a whole post about insecurity that I fittingly never posted because I worried about how insecure I might sound after posting it... wrap your head around THAT.) I worried about doctor visits, I worried about doing well at my job, I worried about giving Knox enough attention at the side of a brother with special needs, visitors, and doctors; I worried that, for the first time in my life, the things I really, really, really wanted for Christmas couldn't be wrapped and put under the tree, and for once, I didn't know if I'd ever get them. I worried about challenging and engaging Hayes enough, I worried about being a good mom and a good person.

But the pendulum would swing, and I would find my patroni*.  My faith would restore and arm me.  I felt reassured in knowing I will never been given more than what I can bear, and indeed, I have been given wonderful people and resources to give both of my children happy, healthy lives.  My husband would encourage me to love myself and thus, to love those around me better.  My children are beautiful spots of bright light.  Strength in numbers came in the strangest of places - Facebook (yeah, I know) groups where I connected with parents of other children with ONH and hypotonia gave me (and still give me) incredible comfort and encouragement.  And finally, my family, specifically Koby's and my parents, gave us tremendous support during the past year.  Without their support, both emotionally and financially, the first six months of 2013 would have been very, very bleak .

And so we end on a positive note.  I don't suppose I have a New Year's Resolution (except to be less didactic in all my posts, heh) but to keep the dementors, new and old, at bay in 2014.  To surround myself with the people in the paragraph above.  To remember to be intentional, proactive, and good in what I do and say.





...

It occurs to me that all of that may not read as 'happy' as I mean it to sound, so I think we'll end on every one else's year recaps, in my own words, since they are all unable in their own ways to contribute at this moment.

Koby, who is currently hunting with all the men in his family on a ranch 195 miles away, had another great year in sports - his Varsity football team, on which he coaches the running backs, won district and lost a heart breaker in the first round of playoffs.  Especially fun and exciting for him was the fact that one of his athletes rushed for 1,713 yards this season and scored 21 (!) touchdowns, receiving honors such as the Times Record News' Red River 22 Small School MVP.  He shot two good bucks and a doe during this deer season and was able to squeeze in a good duck hunt, too.  He's still doing European mounts for other people, which is a type of taxidermy-ish thing.  He inspires me as a teacher/coach, because even though we have different teaching styles, I can tell that his students and athletes appreciate, respect, and respond to him.  His kids are always inviting him to go hunting with their families on their places.  He inspires me as a human because he gives selflessly and works hard.  Koby has set a New Year's Resolution to achieve six-pack abs ASAP, so I'll keep you posted on how that transpires.  As we were watching ESPN on New Year's Day, the telecasters were assigning resolutions they deemed appropriate to various NFL entities.  I would like to play that game and assign Koby the following resolution: Become the small-time celebrity chef MAKING the cooking videos on YouTube, instead of just watching them alllllll the friggin' time.  He could totally do it.



Knox, who is currently trying to push a large toy truck under an ottoman (it won't fit, because, physics), has had an incredible year of growth.  2013 began with the flu and a very limited vocabulary and ended with me begging him to please stop talking in order to take a nap, to which he replied "I don't want to stop talking, I want to talk!"  Right now he is enamored of the words 'never' and 'gorgeous', but he hasn't got a handle on when/how to use them yet.  Yesterday I walked in when I heard him stirring from his nap to find him sitting in bed, saying to himself, 'I never? I never. I never.... I never?'  Koby and I are still admittedly obsessed with just about everything he does and says.  Knox is officially, though unadmittedly at this point, obsessed with Pixar's CARS and evvvvvvverything about it.  We are trying to wean him off of iPad time, because right now his favorite thing is to

  1. Find YouTube 
  2. Find movies about the Cars movie 
  3. Watch anything having to do with the Cars movies, including but not limited to 20 minute videos of hands taking Cars toys out of packages, assembling them, and playing with them, and equally long videos of hands c o lo r i n g Cars coloring pages.  

Disney, we have a problem.  Because he has little Cars toys of the various characters, he is beginning to realize that, in efforts to make money, other companies may have also made toys for each of the movies he likes to watch on Netflix.  Last night, as we wrapped up The Many Adventures of Winnie the Pooh for the nth time, he cried -as though it had occurred to him at that very moment, which it had- "I want my Tigger toy!"  We just so happen to have a toddler-size Tigger toy in his bedroom.  Then, "I want my Pliglet [sic] toy!"  We just so happen to have a 'Pliglet' in our second living room.  Then, "I want my Pooh toy!" We do not so happen to have a Pooh toy.  I informed Knox of this discrepancy.  He squinted his eyes at me (am I really this dense?) and encouraged me to "look again" in his bedroom.  Hashtag spoiled.

My resolution for Knox, though I am obsessed with everything he says and does, is to go pee and poop on the potty in 2014, so I can become obsessed with that.



Hayes, who is currently half-heartedly napping on the world's most comfortable blanket (hashta jealous), also had an incredible year of growth.  2013 began with RSV and him essentially not having met any of his milestones, and ended with a mucous-y cold and him rolling over both ways, tracking objects visually sometimes, bearing weight in his legs sometimes, having great head control, and sitting with assistance.  Hayes reminds us to celebrate him on his own time.  For example, a few days ago he held a rice snack thing in his hand and chewed on it.  At the same time.  We exclaimed and took pictures like he'd just signed a contract to play quarterback for the Dallas Cowboys (I heard they needed one anyway) because in our house, Hayes doing that is a BIG. DEAL.  I think his timeline makes us better versions of ourselves as parents and teachers.  He's seen a physical therapist weekly since May of 2013, a speech therapist monthly since ... I can't remember when, a vison therapist, and an ECI case manager and a DBS coordinator too.  He still loves 'rough play' the best ... which sounds weird but essentially just means he likes it when we tickle him or make him 'bop' himself with this hands or clap his hands together.  Right now our focus is to build his trunk strength so he can sit up by himself and encourage him to reach for objects/people/food.  He does well holding his bottle about 80% of the time, which is a huge improvement from about 0% of the time less than four months ago.  On Monday (today is Saturday) we go back to his ophthalmologist to see if, with the vision that he does have, he may be near or far-sighted, in which case he might need glasses.  (Hopefully) soon we'll get his hearing evaluated.  He's grown four big teeth in the past few months and has not enjoyed that tremendously.  Our DBS coordinator is working on getting a SPIO vest to help him work on that trunk stability and so my resolution for Hayes is to keep working hard, to sit all by himself, and reach for things in 2014.




Thanks for listening, for the encouragement and faith you all give me!  HAPPY NEW YEAR.  Let's make this one good.

* Uh, to understand this post you need to have a working knowledge of some Harry Potter vocabulary words.  I didn't want to post this at the beginning and deter some of you Harr-o-phobes from reading.  A dementor is a soul-less creature who survives by feeding off the joy and happiness of others.  A patronus (pl. patroni) is a charm that repels dementors and is created by thinking of the happiest/most hopeful moments of one's life.

Tuesday, November 5, 2013

Hope Ambassador

A few months ago I posted an original design of mine that was given as a gift to a couple getting married in South Dakota.  I don't know them, but my in-laws do; the bride is from the States, and the groom is from Haiti.  They met while the bride was on a medical mission trip to Haiti.  To me, the quote was lovely and fitting for their marriage.  Here it is if you don't feel like clicking over (and also, I misquoted the phrase in the artwork and it is k i l l i n g me):

“Differences of habit and language are nothing at all if our aims are identical and our hearts are open.”




Maybe you were blessed with a spouse who is a lot like you.  Maybe you're surrounded by friends and family who are like your little clones, replicating your personality and beliefs.

Koby and I were not.  Koby and I were blessed with spouses who challenge and counter nearly every point of our personalities.  We are not alike.  Even though we come from very similar upbringings, even though we were raised in the same church, even though we have the same eye, hair, and skin color,

sometimes it feels as if WE may as well speak different languages.  (Married people, can ya feel me?)

Here are some things we've had different opinions about lately or ever: the Dallas Cowboys, the appropriate amount of red meat one should consume, the extent to which the Founding Fathers have been deified, best teaching practice, the environment, Miley Cyrus, the appropriate way to voice political beliefs, where to put the second Direct Tv receiver, bedtime rituals in general, how often Knox should brush his teeth, the extent to which Knox should be clothed when he goes to bed, where to put the soap in the shower, appropriate topics in public . . .

Most are trivial.  Some are not.  Sometimes our different natures work incredibly in our advantage; when Koby is stressed, I remain calm, and vice versa.  Sometimes our disagreements leave us laughing, sometimes they jar us off course and we spend a few angry minutes cooling off in separate rooms before we can realign our marital compass.

But 'we' work.  (And please know, it takes work, as I think any marriage should.)  And I think Mrs. Rowling was on to something when she noted that differences matter not when you're supportively working together towards the same goal.

Today I took Hayes to his neurology check up.  The doctor was very pleased with his strength and progress, but he was concerned about some of the cognitive (mental) delays that Hayes is still showing.  For example, the fact that he isn't supporting himself with his arms while sitting or catching himself when he topples over isn't solely related to his vision impairment.  (Completely blind babies should be able to sit unassisted by 12 months.)

It's worrisome to think about Hayes having some degree of cognitive impairment (mental retardation), but it's not something that was out of my scope of considered possibilities.  It's not something fun, to have the doctor echo what rattles around in your brain in your weaker, doubting moments.

But it's irrelevant to worry because I left the appointment with yet another "We'll just have to wait and see," because that's exactly what we'll have to do.  The realm of possibilities is thankfully and frustratingly endless when it comes to Hayes and his condition - but couldn't the same be said for every child?

And I've realized that God has given me an incredible weapon and force to combat worry and anxiety about Hayes AND to empower our son to achieve at the best of his ability - and that is that I've been blessed with a different habit-ed, different language-speaking, open-hearted, aim-identical husband in Koby.

God can work through our marriage to give Hayes (and Knox!) the BEST possible life, whatever that may entail.


Hayes's AWESOME first birthday was this past Saturday (if you haven't read Koby's birthday post for Hayes yet, check it out), and at the party, I was struck (for the jillionth time) by something else.  God blessed Koby, Knox, Hayes and me with different habit-ed, different language-speaking, open-hearted, aim-identical hope ambassadors in the form of family and friends.

How have I been so incredibly blessed to have so many people who make it a point to strive for the best lives for my own children?






Finally, I want to end with a wonderful verse that gives me comfort and reminds me that not only do we have hope for eternity, but that we've also have hope for the present day, and it has the power to permeate and radiate in our lives now.

But in your hearts revere Christ as Lord. Always be prepared to give an answer to everyone who asks you to give the reason for the hope that you have. But do this with gentleness and respect . . .

- 1 Peter 3:15