Tuesday, May 29, 2018

CVI 101


I recently shared that our baby girl (who was born with congenital hydrocephalus) received an added diagnosis - one that moves us from the "special medical needs" category into the realm of parenting a child with a disability. Of course there is a great deal of overlap between these two "realms" of parenting, but I feel the need to make a distinction - not because it impacts our love for our daughter in ANY way, but because each diagnosis plays a very different role, practically speaking, in our day-to-day lives.

Let me begin by reiterating that congenital hydrocephalus is not a disability; it is a medical condition that can potentially cause any number of physical or mental disabilities due to increased pressure being placed on the brain as it develops in-utero/prior to surgery. The outcomes for a child with hydrocephalus depend greatly on the severity of the pressure on the brain before birth (when the child can then have surgery to manage the condition), what part(s) of the brain were the most "squished" while growing, what (if any) parts of the brain did not form properly, etc. So, while Rosalie's hydrocephalus did plunge us into the world of "special needs" parenting, her early intervention needs thus far have been to address developmental delays she can and will outgrow as she gains better head control (hydro kids are born with large/heavy heads that they will eventually grow into, but the size can obviously delay many developmental skills).

So what is this disability I'm talking about?

Well, a visit to a pediatric ophthalmologist revealed to us that Rosalie has Cortical Visual Impairment (CVI). CVI is the leading pediatric visual impairment in developed nations - which you have probably never heard of; literature on CVI has only really come on the scene in the last couple of decades and even teachers of the visually impaired (TVIs) are not necessarily required to have any sort of official training in CVI (something that is s-l-o-w-l-y changing and needs to improve ASAP!).

What is CVI exactly? 
It is a visual impairment that occurs due to brain injury (in Rosalie's case, being born with hydrocephalus). Rosalie's eyes are perfectly healthy and function exactly as they should; her eyes are seeing, but her brain has a difficult time interpreting what is being seen. Imagine seeing the world as if you were looking through a kaleidoscope coupled with potential "holes" where you see nothing. The world would look like utter nonsense - which is more or less how things appear to a child in the early phases of CVI, although each individual's vision impairment is completely unique to them depending on how/when/where their brain was damaged.

We had anticipated that Rosalie may have vision problems considering they are common with hydro kids and her first MRI at birth revealed that the "vision" part of her brain was under some of the greatest pressure before she got her shunt. However, CVI is not an ocular impairment; it cannot be fixed with glasses or surgery. 

What can be done about CVI? Can it improve (and how)?
Well, since the brain has this beautiful thing called neuroplasticity people with CVI can be "taught" to see by giving the brain appropriate opportunities to "use" their vision in the right context and environment. This is not something that can be achieved effectively with an hour of vision therapy once a month (or even once a week), but requires us to think about and adapt every aspect of Rosalie's environment throughout her daily life. Essentially there are 10 characteristic visual behaviors of CVI and the goal is to help "resolve" these characteristics, at which point a person with CVI could achieve "near normal" vision. Few will ever achieve fully normal vision; for example, a cane may still be necessary for safety purposes if someone with CVI has unresolved lower vision field problems.

So...what next?
The really good news about Rosalie's diagnosis is that not only is it something we can and will do something to improve, but we caught it early enough that we can optimally maximize whatever her vision permanently becomes! Experts tend to agree that while neuroplasticity allows improvements to be made at any age, birth to age three is the "magic" time to intervene and get appropriate vision therapies for CVI. The first year of life is particularly crucial in permanently impacting visual function development within the brain.

The day we got the diagnosis I kicked it into high gear researching. I joined CVI groups online, found the best relevant CVI books, read informative websites and blogs, and more. I quickly learned that there is an expert on CVI (who literally wrote the textbook and developed the CVI assessment tools) and many people fly their kids to be seen by her in Pittsburgh. I also learned that there are currently about 60 people throughout the nation endorsed by this woman (Dr. Roman-Lantzy) and the Perkins School for the Blind to provide the "gold standard" assessment called the CVI Range. I rejoiced when I discovered that one of them just opened a private clinic location practically in our backyard - and you'd better believe that within a week we were on their calendar to get Rosalie assessed! I also made sure that our referral for free (state-funded) vision services went through and had been added to her early intervention plan, so we can get the ball rolling with a teacher for the visually impaired (TVI) that will regularly come to our home. I even found a brand-new CVI podcast, listened to the first episode, and reached out to the host (who happens to live in our area)!

Importantly, I immediately learned that there were plenty of inexpensive things I could purchase right away from a dollar store and/or craft store in order to give Rosalie more opportunities to "use" her vision throughout our daily routines. It's important to get an assessment and learn where Rosalie is at so that we know what interventions to focus on (aiming for things that are too advanced or too easy will not maximize her vision potential), but until then the important thing is get her looking. The more she "sees" the more connections her brain can start to make!

So that's where we are right now. We are quickly approaching both the official CVI Range assessment at the private clinic and the free functional assessment our provided TVI will perform, but in the mean time I've stocked up on all kinds of random items and/or toys that CVI kids are typically drawn to (and I know Rosalie visually responds to). I've integrated these items into our daily lives, which means Rosalie is constantly working her vision (and boy, was she tired the first few days because of it!).

How long does it take to "resolve" all those CVI visual behaviors?
One of the first things everyone wants to know (ourselves included) is how LONG it takes for a child with CVI to be able to see "normally." Keep in mind that a child with CVI may never achieve "near normal" vision. However, one research study performed by Dr. Roman-Lantzy showed that in a select group of children with CVI that had "highly motivated parents," 97% went from Phase I to Phase III (near normal vision) in an average of 3.7 years.

I think it's pretty safe to say that Michael and I fall into the "highly motivated parent" category, considering all that I had read and accomplished regarding Rosalie's CVI within 2 weeks of receiving her diagnosis.... So, perhaps when she is 4 years old we might get to late Phase III?

Needless to say, I've hit the ground running and am proudly wearing my badge that says not only will I be advocating for my daughter within the medical community, but will also be taking on the education system. It's too early to know what ongoing therapies and interventions Rosalie may need when she reaches school age or what path we will take with her schooling, but I'm learning my options and putting things in my tool-belt for future use.

A final note: many of you recall that Rosalie is participating in a research study that examines the impact of stem cell infusions on potential neurological damage repair. This highly-regarded study at Duke is sought after by hydrocephalus parents all over the country, because it is THE one scientific thing we can do to potentially help our kids beyond standard hydrocephalus treatment. Well, now that we know about Rosalie's CVI, Michael and I 100% believe that her CVI improved after her first stem cell infusion in March.

Post-infusion we saw a huge boost in Rosalie's level of interaction and sudden desire to move around - all of which can clearly be linked back to her vision. In hindsight we know that some of the visual behaviors of CVI improved. This is HUGELY promising! Our baby girl has enough cells from her cord blood to potentially receive 3 total infusions and her second one is coming up in a few weeks. Please join us in praying that the future stem cell infusion will help repair more damage in her brain, particularly as it relates to her CVI. Any boost can potentially help her move to Phase III faster.

Thank you, thank you, thank you for your continued prayers! We seriously know they are helping our little girl and the entire family.

Want to learn more about CVI? Check out these pages, which also link to multiple additional resources:

Start Seeing CVI
Little Bear Sees
Webinars for CVI by Perkins School for the Blind

"No one ever injured their eyesight by looking on the bright side."

Wednesday, May 23, 2018

Motherhood as of late


It's been awhile since I've untangled my thoughts in this space, which basically means I have been continuing to learn our family's new normal with three kids ages 4 and under while performing constant behind-the-scenes work that is typical of any special needs mom. Writing necessarily falls on the back-burner when I'm spending almost every spare minute (when the kids are asleep) researching, advocating, and making phone calls (related to early intervention therapies, medical appointments, medical billing errors, etc.).

Ever since Rosalie's 4th brain surgery back in February (due to a shunt malfunction) we've been basking in the glory of a much-needed respite from hospital life. We even got to celebrate her 3-month "shuntiversary" a couple weeks ago, which is the longest she has ever had a shunt remain complication-free! Having things good and stable with Rosalie's shunt allowed her to finally receive her first stem cell infusion (you can read about the research study we are participating in here and here) in early March and truly, she has been thriving since. We have begun to see big strides with her physical therapy and have seen her exhibit a much stronger desire to move around (she can now get onto both her sides and rolls easily onto her stomach from her left side!) and much more.

I had grandiose plans of writing something poignant for Mother's Day about this journey that I've been on the past several months since our beautiful daughter was born, but honestly...it was just too hard. That might sound strange because I have been blessed with 3 precious children that absolutely make me feel like celebrating this gift of motherhood, but the entire week leading up to Mother's Day had me feeling the weight and depth of the heavy waters I have been plunged into this past year since we first learned of Rosalie's prenatal diagnosis.

I saw a handful of lovely Mother's Day videos circulating around, but this is the one that I identified with the most this year:

And this Mother's Day article about remembering the mothers of sick children (seriously, click on over and read it - I'll wait) made me tear up while nodding along, because it did a beautiful job summarizing some of the crosses I now carry while simultaneously articulating the depth of my joy as Rosalie's mommy. It also explains that my abilities as a mom to a child with a brain condition are not superhuman; I just intensely love my child(ren).

I realize that I sound like a broken record, but I need to continue saying and acknowledging that Michael and I have just lived a LOT of Life this past year.

Not only was Mother's Day right around the one year anniversary of our ultrasound when we learned about Rosalie's hydrocephalus (making me all kinds of pensive about what God has challenged and abundantly blessed us with ever since), but the week before Mother's Day we received an added diagnosis that means we are now parents of a child with a disability, which is a lot to mentally process.

Now, before I continue I feel the need to clarify that hydrocephalus is not a disability. It is a medical condition that can potentially cause any number of physical or mental disabilities before surgery takes place OR as the result of shunt complications, but in and of itself it is not a disability. Because hydrocephalus puts so much extra pressure on the brain (particularly when it is forming in-utero) the outcomes widely vary and not everything will be detectable at birth; some things may take months or years to manifest - like the fact that hydro kids have a lifelong risk of developing seizures (it may happen, it may not).

Thankfully Rosalie's recent diagnosis (which I will elaborate on soon) is one that can greatly improve with proper intervention - especially when it is caught so early (praise!). Time is of the essence, so on May 7th I really hit the ground running. I kicked it into high gear with researching and doing what I needed to do in order to help Rosalie immediately - and all this was in the days leading up to Mother's Day and, shortly thereafter, the anniversary of our life-changing ultrasound. Basically my mental load was huge, which is the main reason I finally prioritized putting some of this down in writing.

It's only been a couple of weeks, so most of my family and friends probably have no idea that I have practically fallen off the face of the earth. Sure, I've intentionally gotten out of the house with the kids, scheduled some play dates, maintained our daily routines, and tackled any mandatory responsibilities, but when a friend asked what I've been up to and I responded with, "researching," boy, did I mean it. My husband and my mom gave me Mother's Day gifts that included 3 (somewhat pricey) medical and educational books regarding Rosalie's new diagnosis because that is the only thing I wanted.

Well, I wanted the books and I asked Michael to teach the boys what Mother's Day is - because I'm typically the one that prepares them with explanations for holidays and celebrations and I refused to do that for Mother's Day this year. ;) The way the boys greeted me in the morning with hugs, "Happy Mother's Day," and Gabriel sweetly told me he likes it when I take him to swim class demonstrated that Michael followed through on that request. :)

So, that's what is new in our neck of the woods! A whole lot of reading, thinking, praying, being thankful for my 3 amazing kids, and incorporating new things into our daily life with Rosalie. We love you so much, baby girl!

2&3 Photography

As always, thank you for your prayers (we know they are helping!). I hope all of you had a blessed Mother's Day!

"The Most Important Person on earth is a mother. She cannot claim the honor of having built Notre Dame Cathedral. She need not. She has built something more magnificent than any cathedral -a dwelling for an immortal soul, the tiny perfection of her baby's body. . . The angels have not been blessed with such a grace. They cannot share in God's creative miracle to bring new saints to Heaven. Only a human mother can. Mothers are closer to God the Creator than any other creature; God joins forces with mothers in performing this act of creation. .. What on God's good earth is more glorious than this: to be a mother?"
-Joseph Cardinal Mindszenty