Sunday, May 17, 2015

An Update on Zella

Being the fourth child, Zella has probably been ignored the most in her first four short months here on earth out of all our kids.  She takes it all in stride, however, and really enjoys watching and listening to her older siblings fight play.

On Monday, May 4th, three days before I turned 30, I took Zella to her 4 month well check at her pediatrician's office.  I had a couple of questions to ask about some strange things we had seen and Jake was kind enough to email me a list, so I wouldn't space out and forget.  Naturally it was shorthand doctor-speak...errr...awesome.  So when the doctor came in, I rattled off the list and handed Zella over, fully expecting her to say "nah, no worries here."  Much to my surprise, our pediatrician was also slightly alarmed at some of the same things Jake had noticed.

Primarily we noticed that both her eyes seemed to dance back and forth horizontally whenever she would try to focus in on something (nystagmus).  I had also noted early on around the 2 month mark that each of her pupils were slightly different in size (anisocoria).  And lastly, she seemed to have a lazy eye that continued to persist beyond three months, causing her eyes to look crossed (strabismus).  Technically a couple of these things can be fairly normal and harmless, but Zella's nystagmus was really what concerned both Jake and her pediatrician.

Our doctor made an appointment with a pediatric ophthalmologist for two weeks from that day, the earliest available time, and consulted with a neurologist about her case in the meantime.  The pediatric neurologist thought it would be worth it to also order an MRI just to see exactly what they might be dealing with.  We were *very* lucky to have an MRI opening come available due to a cancellation two days later, and so we took it.  Her doctor also ordered an ultrasound for the following day to rule out any abdominal masses, which might be a sign of neuroblastoma.

The ultrasound went well and our doctor called us with the results that same afternoon.  No masses!  We still didn't have any answers as to why she had these slight eye abnormalities, but we knew it was important to continue ruling things out as quickly as possible.

The next day she went in for the MRI, which was tolerable, but not great for Zella as they needed to put her under general anesthesia in order to administer the test.  This meant nothing to eat for over 6 hours, and clearly she wasn't thrilled about that.  We got the MRI results back 48 hours later and were very relieved to see that again, there were no masses.  The radiologist did, however, find something strange.

On Friday, May 8th we were told by our pediatrician that the radiologist had noted mild to moderate widening of her ventricles, not caused by hydrocephalus.  He mentioned that this thinning may have been caused by some sort of "insult" to her system.  The current theory is that while in utero, whether early in the pregnancy or during labor and delivery, there wasn't sufficient blood supply to part of Zella's brain.  There is no answer as to why this happened or when exactly it might have occurred, but the results show that *some* of her brain tissue became necrotic and died as a result of this insult.

This was a lot for Jake and I to take in.  I know...understatement of the year.  We don't yet know her prognosis, because as with many things, it is a "wait and see" deal.  At minimum, she might be completely unaffected thanks to the plasticity of children's brains.  At maximum she may get a cerebral palsy diagnosis sometime between the ages of 3 and 5.  We have no idea.

The craziest part of all of this is that Zella's nystagmus, the reason for all of the diagnostics, can not be explained by this insult and subsequent widening of her ventricles.  The neurologist believes that the two issues are completely separate.

This past week we took Zella to the pediatric ophthalmologist where he asked us how well we thought our daughter could see.  I mentioned that compared to the other three kids at this age, she doesn't seem to be able to focus in on your face nearly as well.  He told us he agreed that her vision is definitely diminished.  It was then that he dilated her eyes and examined the retinas.

The ophthalmologist found that Zella doesn't have an adequate foveal pit, a condition called foveal hypoplasia.  Generally this is associated with albinism and ocular albinism, hereditary conditions that do not run in either of our families.  Zella will have "mild to moderate vision impairment that can not be corrected with surgery or corrective lenses," the specifics of which we will not know until she is of an age where she can tell us what she is seeing (age 2 or 3 or even older).

The foveal pit essentially seems to control/facilitate our vision acuity and how well we see fine details sharply.  I have been told that Zella is most likely seeing everything similar to how we might see things in our peripheral vision.  It is sad for us to contemplate Zella not seeing everything as clearly as we do, but I know that she will never see it that way as this has been the only way she has ever looked at the world...a blessing in and of itself.  I also wonder if she will be able to read music and be in an orchestra, go to a regular school, or even drive one day.

For now we wait and watch with the help of pediatric ophthalmologists and neurologists, but mostly we pray.  We pray that the great physician will heal Zella while here on earth, if it is in His will.  We also hold firm in the knowledge that Zella and all of us will be made whole one day in eternity.


Holly Coltea said...

My sweet friend. We have missed you so much in Nashville. I am thinking about you right now and praying so much for you and for Jake, as you parent your other 3 kiddos and attend to Zella at the same time. Sending you a big huge hug. xo, Holly Coltea

Amanda E. said...

Thanks so much Holly! I'll be at barre tomorrow morning (after a HUGE slacker break, hahaha) - can't wait to see you all!