Emily is 6 months!

Our sweet girl is now 6 months old, 20 pounds and almost 27 inches long! As cliché as it sounds, time has gone by too fast. I enjoy watching her grow, but I wish I could pause time for just a little bit.  

We finally got our referral for vision therapy approved by the neuro-ophthalmologist!  The structures of her eyes still look healthy and her nystagmus seems to have resolved.  Emily was diagnosed with intermittent slight exotropia, which means her eyes occasionally drift outwards.  However, I haven’t seen her eyes do that in a long time so I’m not convinced.  The neuro-ophthalmologist said she saw her left eye do it once during the exam, so we’ll agree to disagree.  They aren’t doing anything about it at this point anyway so it doesn’t matter – it just adds to her long list of diagnoses.  The main takeaway from the appointment is the diagnosis of Cortical/Cerebral Visual Impairment, or CVI.  Just like the seizure diagnosis (which ended up getting retracted), it made this mama’s heart a little sad, which I tell myself is silly because I’m not surprised.  I could have told you that she had it before we even had the appointment.  I’m trying to prepare myself for a lifetime of diagnoses secondary to the hydrocephalus, but is that something you can even prepare yourself for? 99% of the time, I feel we live a “normal” life and I embrace everything that Emily does or doesn’t do.  But that 1% of the time, I feel cheated, desperate to fix something I can’t fix, and knocked down.  But as always, I pick myself back up and get back to living this wonderful life with my amazing girl.  I’m starting to think I will always have to deal with that 1%.  

Whew, sorry for the rambling!  Anyway, CVI basically means the eyes work just fine; it’s the part of the brain that processes vision that is impaired.  It’s a complicated diagnosis that affects each person differently and I’m in mommy-research mode trying to find anything I can do to help Emily reach her full potential.  I’m eager to start vision therapy because they will be able to better pinpoint where her vision is lacking and what we can do to help.  Thank goodness for neuro-plasticity – some children with CVI can improve their vision with a lot of work.  Honestly I’m a little overwhelmed with this information because it’s hard to know what/how/where she sees things, and what to do to help her.  The impairment can range from not seeing objects clearly, to not seeing certain objects at all, changes in seeing colors, details, lines, shadows, depth perception, loss of peripheral vision, blind spots, etc.  I observe Emily more than I’d care to admit and I’ve noticed things that are “off.”  She clearly sees faces but I can hold a toy in front of her and it’s as if she doesn’t see it at all.  Sometimes, if it makes noise or moves, she will suddenly “see” it.  But she won’t track it from side to side, a lot of the time.  If the toy touches her chest or hands, she will grab it quickly, as if she didn’t see it before and is using touch to find it.  I don’t understand how she can make such good eye contact with us, but then doesn’t seem to see toys right in front of her, at the same distance our faces are from her.  It’s not that she doesn’t care about toys – she clearly shows excitement when playing with toys and brings them to her mouth.  Also, at this age, children typically look at an object, pick it up, mouth it, look at it again – thus learning about that object and making a schema, or “folder” for it in their brain.  Every object they explore adds and enhances that schema in their brain.  Emily only rarely looks at toys she is playing with.  In fact, as time goes on, I feel she is relying more on touch and sound and her vision isn’t playing much of a part in her playtime.  She also gets easily overwhelmed with many different objects at once, or visual clutter as it’s called in many CVI resources I’ve read.  New experiences overwhelm her, too, and she doesn’t appear to look at much when we’re out and about.  Since CVI is caused by brain damage, it can range in severity from day to day, and different times of the day.  For example, when the brain is tired or focusing on different skills, it can cause the visual area to respond even slower.  I have noticed that with Emily – sometimes she is visually attentive to something specific but then acts as if she doesn’t see the same thing at another time.  When experiencing something new, she deviates her eyes downward and to the left and I think that’s her brain’s way of coping – like it’s too much to handle.  She’s doing it more frequently, and I’m not sure what to think of it.  Moving on from CVI stuff…

To no one’s surprise, Emily loves eating pureed food!  So far, she has had rice cereal, apples, pears, prunes, chicken and avocado.  She seems to like all of them, avocado being her favorite so far.  I decided to make her food at home and I’m enjoying it! Easy peasy and cheaper than store-bought food.  

She also decided to start sleeping through the night all of a sudden, about 2 weeks ago!  With that, we moved her into her crib in her room.  She is doing better with it than mommy is!  I just miss her so much and almost can’t wait for her to wake up so I can sneak her in our bed again.  Pathetic, I know.

Stay tuned for some cute Halloween pictures!

The Brain

The brain can be a mysterious thing...

While we were admitted in the hospital for Emily's EEG, she had a rapid MRI done to check the shunt (standard procedure for any symptom that could be shunt related.)

Our neurologist wanted to go over the results with us so that we had a better idea of Emily's prognosis for development.  First of all, he said that if anyone tells you that you can predict what she will do based on scans of her brain, they would be lying to you.  That being said, it appears that her entire occipital lobe is missing and large parts of her temporal lobes.  Basically the only part that looks perfect is her brainstem and her frontal lobe looks good.  Based on this, you would guess Emily is completely blind.  However, she can clearly see.  In fact, she seems to see well.  We don't know what her brain is interpreting as far as sight and it depends on her being able to tell us to know for sure.  Thanks to neuroplasticity and maybe the cord blood infusions, other parts of her brain have taken over for the parts that are damaged. Her neurosurgeon has a different opinion though- he thinks there hasn't been enough time for the fluid to drain to determine what parts of her brain are missing and/or damaged, hence why he hasn't ordered an MRI specifically for that purpose yet.  He said it will take years before that can be done.  I never would have guessed that doctors could have different interpretations of clear-cut scans, until I was immersed in this world of hydrocephalus.  Doctors also have different opinions on whether her corpus calossum, the small bundle of nerves connecting the two hemispheres, is partially absent, underdeveloped, or just very thinly stretched from the large amount of fluid.  We know it's not completely absent, because you can see a small portion of it on the MRI.  But the part you can see, is thinner than it should appear.  She is also missing her septum pellucidum, which is not up for debate.  This is one of the first structures, along with the corpus callosum to "blow out" when you have severe hydrocephalus, so it is a common finding.  Our neurosurgeon assured us that the septum pellucidum doesn't have much influence on her outcome.

Overall, how a brain looks on a scan doesn't determine how a child will do clinically.  A child with a "bad-looking" MRI can look great clinically, while a child with a "perfect" MRI can be doing poorly clinically and vice versa.

The corpus callosum and septum pellucidum are the two parts in the middle of the brain.

Weekend of Firsts! June 20th - 21st

We celebrated our 8 year Anniversary and Father's Day by going to Wilmington for the weekend.  Emily had a lot of her firsts and would like to share her adventures!

First Father's Day

First time in a hotel

Not a first, but doesn't she look adorable?

First time swimming

First time to the beach

First time touching sand

First feel of the ocean

First trip to the aquarium

First stroll along the Wilmington boardwalk

Update

Welp, I certainly did not mean to take 3 months to post another update… I do have a super cute excuse for a distraction (and endless laundry to do).  A lot has happened in the last 3 months!  Emily is now 5 ½ months old and adding more facial expressions, sounds and skills to her repertoire of cuteness each day.  Every single day, she does something or just looks a certain way and I stare at her and tell her she couldn’t possibly get any cuter – but she does somehow.  I am crazy in love with this girl.  She is babbling more and recently added a gurgly “g” sound to her conversations with me.  Her head control and strength has really taken off in the last month, most noticeably during tummy time, which she tolerates much better now.  She is now picking her head up off the ground and turning it side to side with ease.  Still not keeping it up for long, but her endurance is getting better.  On September 7, she rolled over from her tummy to her back for the first time!! Emily has an amazing physical therapist that she sees once a week– one of her favorite people – that has helped her come this far and taught us how to help Emily the other 6 days of the week.  Her physical therapist says that she is making improvements each week.  Emily might have to work harder to achieve these milestones, but she is tough and stubborn!

Now to the medical stuff:

Emily started having these odd eye deviation episodes that made my mommy instincts, along with reading about other children with hydrocephalus, think seizures.  She would quickly deviate her eyes down and to one side, and keep them there, almost as if they were stuck, with some beating-type movements.  I called her Neonatology team who decided she should be seen soon to get an MRI and/or CT scan to check her shunt.  It wasn’t an emergency but seemed urgent enough that she wanted us to go to the ER the next morning, since that was the only way to get an MRI on short notice.  So off we went.  After describing her symptoms and showing video footage we had of the episodes, to oh, I don’t know, 8? Doctors, we were admitted for an EEG (after the MRI and CT scan showed that her shunt was working properly, thank goodness).  The neurosurgery and neurology team agreed that her episodes seemed seizure like, even though not the typical signs of one.  After hours of waiting, we finally got to our room for the EEG, in the same unit where she was after the NICU. (That certainly brought back a lot of memories.) Now, you wouldn’t think using a marker to put spots on her head would be that traumatizing.  Emily thought otherwise.  (I’m sorry that your first experience with Crayola was that way, honey!) Finally, they got 30 electrodes on our screaming baby and our 24 hour EEG began.  We had a button to push when we saw any abnormal eye movement.  They looked through the results and did see some “seizure-like potential” but no seizures.  Granted, she didn’t have any long episodes like she was having at home.  We were 30 minutes away from being unhooked and discharged when she had an episode, which came back as a “maybe” seizure, prompting them to move us to a live room connected to a 24-7 video EEG.  Fun, fun.  It’s hard to explain how odd it is to have a video following you constantly for 2 days, knowing there is a person behind it, watching our every move, their job to move the camera.  If I picked up Emily to feed her, there was the camera panning out and back in; walking with Emily to get her to sleep, that camera followed.  Since I’m breastfeeding her, they called to ask how I felt about them videotaping it.  I must have lost some modesty having a baby because I honestly didn’t care and told them to zoom on in, if it meant getting the video they needed.  They still were “camera shy” about it at first until Bryan insisted “she really doesn’t care!”  That “person in the sky” as the nurse called it, was great at their job, I have to say! In the end, it was mostly inconclusive.  There was one seizure picked up on the EEG, but it wasn’t when she was having the eye movements.  The neurologist said that seizure was debatable as being classified as one.  He attributed it to abnormal brain activity, which children with abnormal brains are going to have anyway.  Since our case was complicated, the head epilepsy doctor at Duke took a close look at our videos.  During one of her episodes, there was no abnormal activity on the EEG, but he said she had a seizure just based on what her eyes were doing.  We talked medication.  We expressed our concern about side effects.  Our neurologist said he was comfortable waiting to medicate and wanted us to keep a detailed log for 2 weeks, then reconvene.  2 weeks later after watching several more videos of her and reading the log, he was still on the fence.  He said there were many indicators that told him it might not be seizures and just something she does to compensate visually or a behavioral oddity.  After about 2 months of these episodes, they slowly stopped.  She will still do them occasionally but only briefly.  Apparently, she likes to baffle us.  Seizures are a possibility in our future, as children with hydrocephalus are at risk for them.  I guess we’ll cross that bridge when we come to it.

On to lighter news: 

Emily had her 3^rd cord blood infusion!!! The process was exciting, as always, and it was a pleasure to see Dr. Kurtzburg and her team.  It was a struggle to get an IV in our chunky baby – they finally had to do one in her head.  I wish we would have started with that one, since she cried way less than she did while they tried her arms and legs.  Trey, the musical therapist, came to sing to her and even let her touch his guitar!  We were talking about how bittersweet it was, being her last one, when they told us they had enough for ONE MORE!!! That one is scheduled in November, right before Emily turns 8 months old. 

We just had a neurosurgery appointment last Thursday.  The appointment lasted 5 minutes and cost  $40 but it went great!  Dr. Fuchs was thrilled with how healthy she looks, in particular how chubby she is now (19 pounds, 2 oz!)  Her shunt and soft spot felt perfect.  He was going to do another rapid MRI in December but he decided to wait until March, when she is 1 year old.  I asked if he was going to do a more detailed MRI where she has to be sedated but he said maybe at some point, later down the road.  My nosy, detail-oriented self wants to have one done so we can have a better look at her brain but in the end, I do agree with him that if it doesn’t change the way he would treat her condition, it isn’t worth the sedation.  We know by now that it doesn’t matter what her brain looks like anyway, it’s what she can do that matters.  And she’ll show us that on her own, in her own way!

We have a neuro-opthamology appointment on September 24^th. We hope that she will recommend visual therapy.  She denied it once already because she said her exam at 2 months old was typical.  However, Emily is not tracking objects as well as she should be so I hope by telling her that, she will write the referral this time.  Visual therapy can only help her, so keep your fingers crossed that she agrees!  We also have her 6 month pediatrician appointment on September 29^th, where she will get the OK to start solid food (yay!) and see if her PFO has closed yet.  Oh, and shots for the poor girl.  

I will try to update again soon, not 3 months later this time!

EEG baby!

 

After her 3rd cord blood infusion! (Note the band-aid from her IV)

Raising awareness for Hydrocephalus!

Strength Redefined

I don't think there is one true definition of strength. In fact, it's hard to describe what strength really is. I think it's hard to put in words just like love, instinct or resilience. I don't even think it's a choice. When you feel such ferocious love for your child, the kind that overpowers you, you can't help but feel strength. Day to day, it's easy to forget that my daughter has hydrocephalus. Yes it's physically obvious, but to me, I could stare at her all day and wonder how she could look so perfect. Right now, I enjoy every move she makes, not being surprised at what she does but still not taking for granted that she might not be able to do that. She moves her legs, and I am grateful that she can. She follows me with her eyes and I'm amazed that she can see. The dog barks and I'm so happy that she jumps. I enjoy the simplicity of each day with her and revel in the fact that she acts "normal." 

But the fear is there. The fear that one day, her development will be obviously delayed. That other kids will ask what is wrong with her. That she won't be able to make choices in this large world. That she'll cry in pain and there won't be a damn thing I can do about it. Or that someone won't believe in her. We look at her and think there's no way she will show brain damage because of how good she is doing now. But what if we're wrong? I think about the simple fact that her precious brain is so severely compressed against her skull and how cruel that is. It's not fair. It makes me angry, scared and desperate. To think I would give anything in this world to make it not so, but I just simply can't. My love is immeasurable yet I can't wish that fluid away. 

I've fully embraced this world. But how could I not? It's a family I never thought I would want to be in. I've struggled to find words to explain how it feels to know other hydro parents and just know they get it. I don't need to write this post for them to understand my feelings. That crushing blow when a doctor tells you there is something like this wrong with your child. We've felt it and remember it like it was yesterday.  Most of us have been offered termination and will forever remember that. We'll look at our children and wonder how that could ever have been an option. Not frequently but just enough to know it sets us apart from other parents. There's no other feeling than wondering about the quality of your child's life and knowing the only answer is "wait and see." The "wait and see." That is so hard. The hardest part of pregnancy. But when you learn to let it go, how freeing it is. Not knowing how things will go is the exact opposite of my personality. But I've learned how to do it somehow. I have no idea how. I'm sure there's many hydro parents that have the same personality and we have no choice but to "wait and see." I wouldn't change my daughter for anything. She's perfect to me. But how easy it would be to be naive and expect the same path of typical development. How easy to not have the heart-gripping fear that could sink you if you let it. I think struggles in life are necessary. However, I want my daughter's struggles to be trying hard in sports, not getting her way, learning how to deal with failure. Not waiting for the next shunt revision, feeling less capable than other kids, or basic motor activities. I know the hydro is not my fault, but it makes you feel like a failure as a parent. 

 

But these hydro babies and their parents. Wow, they are amazing.  How each milestone is so precious now. How we as parents, understand it and celebrate right along each other. How much it means to me that you see such beauty and strength in my daughter.  I wouldn't get that anywhere else.  I never thought I would feel so proud for stranger's babies. But I do. I'm happy alongside you and see such beauty and strength in your babies. And in you. It's like I know you. I still wonder how that's possible to feel so connected to others you don't know in person. The strength we have is unique and feels like a secret club that I didn't know existed. I wish I could explain it better. But I don't think I need to.

 

Neuro-ophthamology and MRI/Neurosurgery Appointments

We are elated about Emily’s appointments today!  She passed with flying colors at her neuro-ophthamology appointment – there appears to be no damage or atrophy to her optic nerves and her retinas are nice and flat like they are supposed to be!  She does have a slight end-gaze nystagmus, but that can be very normal in babies her age.  She still isn’t in the all-clear as far as vision; things can show up later, but so far, we are some happy parents!  She will be seen again in 6 months to make sure everything is still looking good.  

Emily was a champ during the MRI!  The technicians warned me that she would cry a lot during the whole thing but she was just fascinated by the sound and only cried at the very end – I think she was just tired of sitting still.  She showed them!  I got to lay down with her in the MRI tube – I wish I had a picture of how cute she was in there.  We both had earplugs and it was still loud.  Daddy had to stay outside the room and he even said it was loud. 

Dr. Fuchs, our neurosurgeon, was very happy about the results of the MRI.  He showed us 2 images and her brain has fluffed out some!  Wonderful, beautiful brain!  There is still a lot of fluid of course, but the shunt has to work slowly so it doesn’t overwhelm the brain.  Sometimes, the brain doesn’t fluff back out or it collapses on itself, which causes fluid to spread on the outside of the brain.  Dr. Fuchs said that’s a sign of the brain not being resilient to the pressure but we didn’t see any of that.  Of course, in my true fashion, I asked a bunch of questions, in particular if the corpus callosum was there.  It is, thank goodness!  However, it is very stretched and thin, so only time (and less fluid) will tell the true story behind it.  !  We also couldn’t get a good look at any other structures for now.  But we did see fluff and folds!  The brain is still at its most compressed in the back, the part responsible for vision.  Since she is tracking objects and clearly seeing us, the assumption is that it is not too damaged. Despite all my questions, Dr. Fuchs said the pressure is off the brain and it is fluffing back out , so that’s all that really matters.  Going from seeing a sliver of brain to what it is now, is beautiful to us!

I wish we had before pictures from her ultrasound to compare.  I'll work on getting those up soon. 
The grey part is beautiful brain and the white part is fluid.

Emily's brain - still a lot of fluid but this looks dramatically less than before.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Normal sized ventricles - the black parts in the middle are the ventricles

Emily's brain - the greyish part is her brain and the white is fluid

Normal brain - not the best comparison but I couldn't find an infant MRI picture of this angle