Soldier on

I felt a familiar dread on Sunday night. It was going to be one of those weeks. The appointments have snuck up on me. She has 4 extra appointments outside the usual 3, plus school. Not to mention the trip to Salt Lake City (for Shriners and some fun), work, basketball games, haircuts…

I’m worried about Keira. She has some symptoms that have taken me on what Seems to be a wild goose chase. A whole lot of tests for what I’m guessing will be the MD’s equivalent of a shrug (They use a lot more words.).  

Jeff might be worried, too. After all, last night he dreamed Keira was a refrigerator.

Flipping lazily through my phone Sunday night, I came upon this t-shirt. Jeff and I both laughed.

In preparation for a talk I plan to give on anticipatory grief and coping for families like ours, I’ve recently discovered that the particular grief of parents with medically fragile children has not been well studied. Experts on PTSD, however, have shed some light on these families by comparing them to soldiers in combat. The “trauma”, or at the very least, the intense anxiety that these parents feel on an ongoing basis serves the purpose of keeping them alert and ready for the next inevitable, albeit unpredictable crisis. For soldiers, and in many ways parents, the hypervigilance, sleeplessness, planning, and advocating (fighting), is a fact of life. For soldiers, standard treatment for anxiety is often contraindicated. Their state of being comes at a high cost to their emotional and physical health, but it helps ensure the safety of those around them. I’m not a soldier. Or a superhero. Just a mom trying to get through. And so it continues.

I drew this today. When I get into these moods, I often wonder if I could organize and galvanize my thoughts by illustrating the issues in picture form. This one makes more sense than most I’ve tried before. It shows what I’ve been managing for K during the past week. When I finished drawing all the connecting lines, I handed it to Jeff and said, “This is my tight shipwreck.”

As usual, Keira is ok. Today she correctly identified the requested color two out of two times, ate a piece of cheese the size of my little finger, got poked for her baclofen pump refill with nary a flinch, and smiled at a baby in the elevator. All the while worrying me with her increasingly glassy stare, flushed cheeks, and stuffy nose. 

Keira's Christmas Showcase

We were looking forward to this years "Christmas Showcase" more than ever. Last year, we opted to have Keira participate, but we weren't sure whether she'd enjoy herself or steal the show by retching and vomitting. I'd hung a small string of lights across the tray on her chair. By pushing a button, she was able to make the lights flicker on and off, giving her a "part" while the other children sang. It turns out she was delighted. Not only did she enjoy herself, she held up her arm and stuck out her tongue. More please! I want to play! What else can I do? 

More excited this time around, I found a longer strand of lights and wove them through Keira's chair so that they could stay put for the remainder of the season. The video shows her proudly trying them out. They are attached to the yellow button by her head and stay on for as long as she is holding the button down. 

At the showcase, the kids had to arrive 30 minutes early. I figured I'd stay with Keira, since volunteers sometimes look like fish out of water if they aren't familiar with her. I wheeled Keira into a classroom, where a few kids had already arrived and were quietly coloring at a table. Immediately, I hear, "Keira's here!" and before I know it, Annie has taken Keira from me and pushed her chair over to the other kids! Slightly taken aback, I count my blessings and remove myself to go find a good seat in the sanctuary. 

I take a seat with Jeff and the boys, where I sit semi-anxiously, worried that we've sat too far back, where it will be too obvious if and when I have to swoop into the performance to help Keira out. 

The kids enter the sanctuary. The "big" kids (kindergarten-6th grade) sit near the front to watch the preschoolers perform and await their turn. Annie wheels Keira up to the edge of the pew next to a row of girls and sits down next to her. It isn't long before I can hear Keira squealing with laughter. It makes my heart so happy. But she keeps getting louder. She'll quiet down when the music starts, I hope. A couple of girls ask the children's' pastor if they can sit on the floor next to Keira and she approves. Keira and a group of 4 or 5 other kids. Just giggling and laughing. 

The program begins. The congregation sings. The preschoolers do their part. And all throughout, the music is punctuated by Keira's happy squeals. I turn to Jeff, "Should we go tell her to be quiet?" We are actually talking about reprimanding our daughter! "Just let her be," he says. 

It's transition time and the big kids go up on the platform and find their places on the risers. Liam and Jeff hoist Keira in her chair up onto the platform, right up next to the risers. The children begin to sing. Keira cranes her neck, looking to see her friends who are standing next to her, but up on the top step. She looks adoringly at her friends, then brings her head down to smack her lights on and off with gusto. Off and on. On and off. Watching her friends. She can't get enough of this. She seems completely comfortable up there. Like being on stage, she's in her element. I couldn't be more proud. 

The show is over. A merciful sub-60 minute showcase. No sooner than Jeff and Liam carried Keira off the platform, had her friends run up to Jeff, "Can Keira come with us?" Of course!

There is a cookie reception and as we join our church family in the reception hall, I flit from one conversation to the next, greeting old friends and new. I am scarcely aware of Keira's presence in the room somewhere. Not much more than I'm aware of Liam and Ronin. 

You know how it is. Your kids are comfortable enough with the community, and you are comfortable enough with the community, to loosen the tether and let them range free a bit. You enjoy the moments where they are entertaining themselves, making their own fun. You just hope that their unsupervised cookie eating won't result in some late-night carpet cleaning for you. 

So though I wasn't with Keira, I was relishing this moment for us. And for her. The girls pushed Keira through the line as they got their refreshments and sat her up close to them at the table while they sipped hot chocolate and nibbled cookies and demonstrated for the 27th time how they could make their skirts twirl. When I finally went to check on Keira, she was happy and relaxed, continuing to display her lights whenever they were mentioned. 

I am so happy because she is so happy. I'm so grateful that she can have a real, fun, Christmas pageant experience with other kids her age. Heck. She's enjoying this more than either of her brothers ever did. I'm so proud of the other kids. I'm proud of Keira. Love is a beautiful thing. The fact that you can be just as proud of your kid who pushes a button with her head as you can be for your kid who sings in the choir or the one who has a lead role in the skit. 

At home that night, Keira, exhausted, is tucked into bed. The boys are back at their games. In the living room, I perch on the arm of the couch next to Jeff. "That," I say, "That is what life is all about." 

Bravery

This girl is the epitome of bravery. Strength and courage in the face of scary things. 

Today is surgery number...wait, let me count them...11. 

Sedation number 14.

In some ways, a procedure like this is less nerve wracking as parents than it used to be. Trouble breathing and hospital admission are no longer inevitable. In other ways, it’s more nerve wracking than it used to be. Keira has come to show us that there is so much she understands and in her own way, she is a better communicator. That means she has the ability to understand in advance, to some degree, what is going to happen and in return feel anxious about it. It’s when she gets really upset that we are reminded like a punch in the gut how fragile she really is. 

Bless her heart. This is how our morning went. 

4:50 am: We wake her up and change her diaper. She gets to stay in her PJs. No food or drink allowed, so thank goodness it’s far too early for her normal breakfast anyhow. We load her up in the car. I cover her with an 8 lb weighted blanket, given to us by a friend, hoping this will help sooth her. There’s pretty much only one reason she ever gets into the car when it’s still dark. 

5:10 am: We check into the hospital and find a seat in the waiting room. Dad is here to make Keira smile. We get a well-timed note from a friend, who’s little girl, Annie, loves Keira. She wants to come over sometime soon to read stories to Keira, who smiles when mention Annie’s name. 

6:45 am: We are finished with pre-registration and sent downstairs to pre-op waiting. The staff there is very familiar. We take a seat and Keira starts to look worried. Her mouth curls up a bit like she has a bad taste in her mouth. She looks as if she would wrinkle her nose if she could. Her tongue retracts and her breathing gets loud. 

6:50 am: Called into pre-op, where K gets her own room. As we set her on the bed, she acts panicked. This is a reflex she’s maintained from infancy, spreading her arms and legs as if she’s falling. After vitals, she calms down and seems to like the nurse. She cuddles up in the warm blankets and soaked up the compliments on her hair, her nails, her glasses. We change her into the familiar gown; the purple one with dogs and cats on it. K still looks concerned, but she’s relatively relaxed and smiles as each doctor and nurse comes through in turn. We learn from the physiatrist  that there’s new Botox research showing some negative long term effects on muscles treated with Botox. We decide to go with our usual mode of operation and treat today’s discomfort rather than worrying too far into the future. 

7:30 am: 2 nurses come to take her back to the OR. Unfortunately, they are wearing their surgical caps, but fortunately the sight of them doesn’t make Keira panic like they have in the past. They bring an anesthesia mask that’s been rubbed with grape chapstick to make it smell good. The nurse fetched some Frozen stickers to decorate it. Jeff and I give her one last kiss and away she goes. We’ve stripped her of her glasses and hearing aids and I realize that this is the first time I’ve failed to remember to bring “Lovey,” the stuffed monkey. No matter. She seems like a pro, headed down that hallway without any visible protest. 

7:35 am: Breakfast. A quick date in the cafeteria before going back to the waiting area so as not to miss the reports from the docs as they finish with her.

8:00 am: I’m texting friends and making lists. I start this post. I flip through Vanity Fair and remember that I’d like to go see Joker. 

8:15 am: The audiologist has good news/bad news. Keira’s right ear continues to hold steady. Bad news is that so does the left ear, which continues to show profound loss. We may not even bother with the left hearing aid anymore, as it’s not helping much and emits lots of feedback. Note to self: Keep appointment with audiology to make new ear mold and schedule an ENT appointment to resume talks about cochlear  implantation. 

8:20 am: A lovely friend has offered to bring pizza for dinner and I gladly accept. A fellow waiting-room waiter talks too loudly about how she’s thinking of spending her extra several thousand dollars a month. She’s interested in ministry. The Hall Family is an excellent ministry, I think to myself. 

8:30 am: The physiatrist steps in to tell us that all of the Botox shots went in well. Biceps, thumbs, lower legs, and toes. Note to self: keep next week’s appointment to refill Baclofen pump. 

8:45 am: I send an email off to the Idaho Perinatal Project, inquiring about opportunities for providing CMV education at their upcoming conference. Jeff intermittently shows me dumb jokes on Reddit. 

9:00 am: The orthopedic doctor says all went well. He gave her a block, similar to an epidural, that will keep her numb for much of the day. There were no issues with anesthesia or oxygenation during the procedures. Note: No baths or swimming for 2 weeks. Schedule 2 week follow-up. 

9:30 am: Facebook. Angry Birds. Coffee. We finally get called to go see her. 

9:40 am: Her eyes are already open when we go back and she slowly gives us a half smile. She’s very drowsy and pale, but her vitals are good. 

Nurse: Would you like me to give her some Hi-cet?

Me: No, she had a block, I don’t think she needs it. Can you give her Tylenol? 

Nurse: No, we only have Hi-cet. 

She gets an order and brings us Tylenol. Keira’s mouth seems uncomfortable. Probably a sore throat from the tube they put down it in surgery. We feed her drops of water. Her stomach also seems a little uneasy, so they give her more Zofran. After learning about Keira’s surgery, Annie has sent her a little video greeting. I show it to K and get a half smile. She’s waking up great so it’s not long before we’re getting her dressed. 

10:30 am: We are on our way home! I text the boys that their sister is in good shape. I had told them, “We’ll be gone when you wake in the morning, but if all goes well we’ll be back before you get home from school. If we aren’t home, it’s because K isn’t managing the pain well, or the medicines, or she’s not breathing well, and if that’s the case we’ll make plans.” Business as usual, and they had both declined for me to call them at school with an update.  

11:00 am: She smiles as we pull into the driveway. She giggles when Grandma asks if she wants to snuggle. It’s not long before they are cuddled up in bed and I get to go take a nap. 

3:00 pm: We send a picture off to Annie. Keira overseers the writing of this post, and the boys get home from school.  

All is well and it couldn’t have really gone better. I suppose Jeff and I have a little PTSD from the past experiences. We were ready for ANYTHING. 

Honestly and weirdly, I have the tiniest bit of something resembling disappointment. (What is that feeling called?) Because I was geared up and ready for sh** to hit the fan. Of course,  we wouldn’t have it any other way. Jeff and I will take the rest of the day to decompress and relax. Thankfully, today is another day that Keira is showing us some amazing strength and resilience. 

We are thankful to all the friends and family who rally around us during these repeated experiences. Today, we are particularly thankful for one special little girl who has gone out of her way to be Keira’s friend. Annie, you may never know how priceless it was, on today of all days, for Keira to be contacted in such a “normal” way by a friend. We love you! 

Ourself: Looking for the little girl in a body that doesn't work

Preface
This post has haunted me for years. I’ve written it in fits and starts and never really found a great way to put the quandary into words. However imperfect, the time is now because surprisingly and thankfully I’m seeing Keira’s personality, honest, true, and undeniable, peaking out more and more in such a way that’s alleviating my own internal battle with the issue. Because I feel this struggle is important, I want to put words to it before it’s too distant a memory.

I have come up with a couple of conclusions while wrestling with these ideas. First, the concepts of a) the developing self in a disabled body and b) the particularities of parenting children with severe disabilities are vastly complex and no matter how well I write, the contents probably warrant a dissertation or two. Lucky for you, I’ve written enough of those for my lifetime. 

Second, advocacy is such an important term for healthy realization of both those concepts. Advocacy is a word I knew only vaguely before Keira's birth. Far from inconsequential, her potential hinges on my ability to be a good advocate for her. Generally a positive term, I think an advocate's effectiveness ranges immensely. At best, a good advocate will help to maximize a person’s interpersonal relationships and functional independence. At worst, poor advocate will suffocate a person’s uniqueness and may even exploit her disabilities for personal gain.

Self and personality

What makes a person a person? What is “self?” Simply speaking, it’s the essence of who we are; our tendencies, proclivities, and neuroses. Our tastes, our aversions, our interests, our motivations, our memories, our uniqueness. Nobody can define the self except the person themselves. However, others can identify personality, which is the outward expression of what a person thinks and feels.

But what about when a person’s body is so severely compromised that so very little is physically and verbally expressed? Herein lies so much of what is difficult about our life. What traits are truly Keira? How much of what we attribute to Keira is really her? How often do I attribute my own preferences and anxieties to her? Does it matter? Am I doing a good enough job of trying to decipher who she is? How often have I have assigned something to her because I had nothing else to go on? I mean, how stupid do I look when someone asks me what her favorite color is and I don’t know?

There was never a doubt that she loves this guy!

A while back, I read a book called Ghost Boy by Martin Pistorius. It’s an autobiography about a man who was typically-developing until late childhood, when an unknown illness stripped him of all volitional movement and the ability to communicate. At first, reading his story was really validating to me. We’ve done so well! Keira would likely be a ‘ghost girl’ if we weren't such receptive, active parents. We’ve given her a life. What if we hadn’t been there all along the way, insisting that the therapists and caregivers wait and watch? Teaching them to notice her?

As I continued to read, I experienced so much anxiety that I stopped reading for quite some time. Martin’s family assumed for many years that he was no longer “in there.” They believed their son was gone and was incapable of intelligible thought. How much of what we believe about Keira is accurate and how much is made up? You see, they eventually found that the young man was of average intelligence and when a keen observer finally noticed, Martin was able to get the help he neede to be able to read, talk, get a job, and even publish the book. Oh my gosh. The pressure. I mean, I know that Keira is no Stephen Hawking, but how much of Keira is still hiding in there? If we tried harder, what could she do? How could I possibly try harder?  



It was hard for me to get through the book and cope with those ideas. Because I have some bad days. Some really dark days. I’m not proud of the things that come to my mind:

What the heck am I doing? Dragging my vegetable around from appointment to appointment like it means something. Like it’s worth something to anyone but me, desperately trying to cling to some shred of dignity and hope that this all for a reason and not just a terrible tragic exercise in longsuffering. 


Sometimes we go to public events or birthday parties or other places where we really want Keira to feel included. Inversely, I feel a responsibility to help others feel comfortable with her, thereby making it easier to include her. I love to take her out, but to go to a birthday party, for example, is a lot of work, with me trying to keep her engaged and helping others to engage with her in nonthreatening (to them) ways. Sometimes I leave those situations feeling like I was working too hard.  I’m not fooling anyone. She’s my puppet; my ventriloquist dummy.  She’s Oscar and I’m the nameless Muppet carrying her trash can around. 

How to grow a self

In what is popularly called the “fourth trimester,” infants are kept close to their parents, as there is little they can do for themselves. We quite literally wear them wherever we go. The baby who was very  literally a part of Mom’s body is now physically existing outside of her, but psychologically, in many respects, is still one with her. That’s why our babies’ cry can trigger the let down of moms’ milk. That’s why the separation of baby and mom can cause mom to feel real physical pain. We use our own experiences to care for our little ones. I’m cold, so she must be cold. He’s just sitting there and that would bore me, so he must be bored.

This connectedness is reflected in our language. Ever notice how moms of littles often use the word “we” rather than referring to herself and her child as individuals? Whether talking to the baby or an outside observer, it’s often, “We have some work to do,” “We have a messy diaper, or “We’re taking a bath.”  Mom may be literally referring to the baby taking a bath, for example, or mommy taking a bath, or both taking a bath together.

This language and sense of connection changes gradually as the typical infant grows into a toddler and starts to do things on his or her own. He develops independence enough that he is doing things that Mom doesn’t necessarily consent to or want to participate in and as the “we” becomes mom and daughter or mom and son, so we recognize the tendencies, preferences, and initiative of the child start to grow. A little budding self. 

Around age 2, our kids typically stop being completely dependent and perhaps it’s that stark contrast from babyhood that makes the twos seem so “terrible” to many. All of a sudden, he doesn’t want to be a “we” anymore. He wants to start his own bath, eat his own food, pick his own clothes. Kids start to play, and through the play, they assert what they are interested in, what they think about, and how they feel.

It's exhausting as a parent to feel like you are never ever progressing to a new stage.Christmas after Christmas, I grow weary of walking down the same toy isle, the one meant for babies and toddlers. I’m hoping for something new that Keira will really enjoy and react to, but I know that I’m mostly going through the motions, because what would her brothers think if I didn’t have any presents for her under the tree?

In many ways, Keira is still an infant. For one, she doesn’t play. At least not independently. She shows very little interest in toys at all. She can’t play with any toys without me directly manipulating them and her. She sees very little without me showing it to her. She doesn't know what is in her backpack when she comes home from school or what toys live in her closet.  So, when she “plays,” her caregiver has usually chosen the setting, the toy, and the manner in which the toys are articulated. I put a lot of pressure on myself to go through the motions and offer her these experiences, assuming that they are enriching for her, but I can’t help feeling that I am putting a lot of “words in her mouth” by putting her through these scenarios.

"Keira's" painting

Some measure of “putting words in her mouth” is completely necessary and important to being Keira’s advocate. I can read her body language better than anybody. So, for example, when she is in the hospital, I am better able to assess her pain level than the average doctor or nurse. But putting actual words in her mouth has always made me uneasy.

From a very young age, we’ve worked with Keira to teach her to use switches for a variety of reasons. She has switches on which we can record speech so than when she pushes the button, she is “talking.” For example, when we are having friends over to the house, sometimes we’ll set up her switch to illicit one of the following with each push of the button: “Hi, I’m Keira!” “Thank you for coming to our house!” “Do you like my new wheelchair?” “Will you play with me?” It’s an exercise in helping her understand communication, to help others relate to her, to help her practice some sort of volitional behavior. In this case, putting words in her mouth seems to be the lesser of two evils. 

Herself 

What traits have been assigned to Keira? At some point, someone somewhere decided that Keira preferred yellow. Similarly, someone somewhere asserted that Keira liked pigs over other farm animals. What I suspect those people didn’t understand back then was that it was very difficult for Keira to visually focus anywhere but her right field of vision. So, when they held up 2 items, Keira wasn’t “picking” the one on the right because she liked it. She “picked” it because she could see it. Yet, the item on the right became her “choice” and her “preference.”

Satisfied with her Hatchimals

We ran with the pig idea. We thought it was cute and we briefly entertained getting teacup pig for her as a pet. We dressed Keira like Peppa Pig for Halloween, which was fun because when Keira's happy she actually makes a snorting noise. I’ve made a point of rewarding her (is the reward for me or for her?) with a little trinket after each of her monthly blood draws. I was routinely buying her mini Peppa figures until we had about 6 or 8 of them. I’m pretty sure I enjoyed collecting them more than she did. I was thankful and proud of her, though,  when she finally asserted (by looking to her left) that she would prefer a Hatchimal over another Peppa toy. That’s Keira in there!!

Never in a million years would I have predicted that I would care so much about my daughter's looks. I have found myself immensely glad that she is cute. That she has pretty curly hair. That she tolerates me dressing her stylishly. That she lets me paint her fingernails. But what I find, for better or worse is that these things add up to 1) making her more approachable, 2) helping others feel more comfortable with her, 3) giving people something to talk about besides her disability. Is it because I value outward appearance so much? Absolutely not. It’s because it is so difficult to know who she is and what she likes so it’s another effort on my part to make these statements for her. Does she LOVE having her nails done? Maybe? She tolerates it. What I know for sure is that she loves attention from others. (Again, if she were typical, I really doubt I would celebrate her trying to draw attention to herself in this way). On bad days, I wonder if I’m just playing dress up with my doll.

Ourself
Is there such thing as “ourself?” I didn’t think so, but I did find it in the dictionary. Maybe it’s applicable to identical twins? Maybe it’s a hoaxy sort of concept for those with ESP or fortune telling abilities? In any case, it doesn’t generally seem that a shared self would be very healthy. Think of terms like enmeshed, conjoined, symbiotic, interdependent, codependent, and diffuse boundaries. None of those are typically used when talking about healthy parent/child relationships. Parent/child differentiation is what is important. But how much can Keira “differentiate” when she cannot so much as get out of bed without me? With Keira, symbiosis and dependence seem like a necessity. Which, in turn, makes me cringe at the idea of  me potentially having to live life without her in the future.

"Our" Halloween costume

It’s not just me sculpting Keira’s identity. She has surely sculpted mine. My time. My professional practice. My writing. My availability to other family members and friends. Being Keira's advocate has become a heavy responsibility that extends outside of our own family and into the community. I’ve written before about how she “makes me special.” The things that I am often known for these days are the roles I’ve been introduced to as a result of my experience mothering Keira: CMV advocacy, parent mentoring, and inclusion ministry. My life now is so inextricably entwined with her and everything she represents. It’s messy. Having Keira has given me opportunities that make me feel important and fulfilled. I’m no longer me without her. She’s not her without me. I suppose that’s true of all mothers with their children. But it feels so much weightier with a child who is so vulnerable.  

Eventually, most kids will clearly confirm or deny whatever traits we have attributed to them. With Keira and kids like her this rarely happens. I keep having to do all the things. I keep having to make all the decisions, claim all the preferences, and magnify any efforts at self- assertion and communication that I think I may have possibly kind of, sort of seen. My job, as I see it, is to identify, as correctly as I can possibly muster, her distilled personality and share it with others. 

K's new glasses are MY favorite color, but she did seem to prefer them!

Keira herself
Just when all this was starting to weigh heavily on me, Keira went through a seriously assertive and sassy phase. I found her often saying no to me (pursing her lips), and only me, much of the time. She seemed to me to be saying, "Take me here. Take me there. Do this for me. Do that for me. But don’t talk. Stay out of the way. Leave me alone." It was as if she were trying to create her own space and semblance of independence. I was proud of her, really, though Jeff did give her a stern talking-to about being respectful to her mother.

Keira is 7 now. Seven, of all the years so far, has seemed to bring with it such unbelievable change. It is hard to describe how a girl with so little functional ability can grow and mature, but she is. Like a flower that grows through a crack in the concrete, it’s hard to imagine that anything could develop within such a confined space. And boy, I'll be the first to admit I’m cynical, but I sure love for her to surprise me.

More than once, by coughing or pursing her lips, Keira has told me to go away when a favorite caregiver comes over. Similarly, she has told me what outfit she doesn’t want to wear. When we ask her about her day, we aren’t just hearing ourselves talk anymore. When we guess at what she has been up to all day, she laughs (accurately, we later confirm) when we correctly identify what she did (ie. Therapy, swimming, painting, or library). As more of her life is lived outside the house, we are finding that others are drawing similar conclusions about what she likes and dislikes.

We have found that when we have gone with our hunches in regards to her preferences, we are usually rewarded. In this way, we decided to have a birthday party for her this year in a fashion that would be suitable to any 7-year-old girl, with crafts and treats and other 7-year-old girls. Boy was that a great day.

Even Christmas feels a little different this year. We’ve decided to splurge and get Keira an American Girl doll, complete with curly blond hair, glasses, hearing aids, and a wheelchair. It’s an impulse for sure, and we know full well that the gift may be more for us (wanting to buy a gift any typical 7-year-old would love) than for her. However, we also have hope that she may enjoy this gift in a way that she's never enjoyed another gift before.

Updates Head to Toe

Hair- Keira has a couple of nickel-sized bald spots on the top of her head; apparently casualties of wearing her CPAP at night. I’ve bought her a satin-lined night cap to wear under her CPAP from now on to prevent pulling, but so far I’m not seeing the hair grow back.

Brain- Last year, Keira was diagnosed with “Continuous Spike Wave Cluster Syndrome.” Never heard of it? Neither had I. Basically, her brain activity is really funky when she sleeps. The neurologist said that little is known about the condition except that kids who are found to have it tend to show developmental regression. So, for that reason, she felt it was important to treat it. Bring on a new medication: Depakote. Interestingly, since taking this medication, Keira seems to sleep more and is more energetic in her therapies in general. Her most recent EEG looked much better, too. Her EEGs still aren’t normal, though we still have never detected an actual seizure and that’s good news.

Eyes- She is still benefitting from glasses. We are so thankful for the developmental ophthalmologist, who practices functional medicine in a way that is altogether different than the ophthalmologist or optometrist. Last week she got a new pair of glasses and she’s looking so grown up. We still don’t know how well she sees, exactly, but we do know that the glasses minimize her “googly eyed” look and seem to make it easier for her to control her eyes.

Ears- My biggest stressor in the past year was probably Keira’s rapid loss of hearing. A key feature of CMV, we weren’t altogether surprised by this, but it still sent me into a tailspin. Anticipating near or complete deafness to come, I spent months worrying about how to maximize her eyesight, learning sign language, learning about cochlear implants, trying to obtain a electronic communication device, and trying experimental treatments. Thankfully, Keira’s most recent hearing test showed that her hearing had stabilized, or in other words, it hadn’t continued to decline. She currently wears a hearing aid in each ear and is taking Valgancyclovir (the same antiviral medication that she took at birth) in hopes of mediating the hearing loss.

Mouth- Keira had 5 teeth pulled about 6 months ago. Because she doesn’t chew and work her mouth the way you and I do, her teeth are likely to resist coming out on their own. Also, pulling some teeth in advance will hopefully prevent overcrowding in her microcephalic (too small) head. She looked so cute without her front teeth, but it seemed that the adult teeth would never grow in. Last week, though, they finally broke through!

Throat/Lungs- Along with the spike wave syndrome, Keira was diagnosed with sleep apnea and we’ve spent the last couple of months getting her used to a full-face CPAP mask. Honestly, it’s gone much better than I’d hoped. Even though Keira never seemed overly tired and didn’t have difficulty sleeping before, she does seem more alert and engaged now that she wears the CPAP at night. 



Stomach- Keira continues to do well with the Nourish we’ve been giving her through her G-tube. She’s been growing quite a lot and seems relatively healthy, overall. We have been so excited to see her interest in eating orally improving. She almost always wants to taste what we are eating at the family dinner table and in therapy, she is working on chewing without a tether! She still is not able to take any substantial volume, but it’s fun to have her socializing with us in this way.

Hips- We went through a spell when Keira’s hips seemed really uncomfortable, particularly in the mornings. Given that she has already had 3 hip surgeries, it was good news/bad news to find out that her X-rays looked clean. The pain may be a combo of growth and the hardware that remains in her left hip from her last surgery. Next month, Keira will have surgery to remove those pins and hopefully that will increase her comfort.

Arms/Legs- K’s arms and legs are perennially stiff. She often wears her clothes to bed as opposed to changing into her PJ’s because it’s just so hard to move her limbs. We rely on the baclofen pump and Botox injections to help with this, but they are both limited. For more than a year we continually increased the baclofen pump dosage, hoping to further improve her tone and discomfort. Basically, doctors suggested that we continue to increase it until we saw negative side effects. Unfortunately, we seemed to reach that spot a few months ago. For the first time, we dropped her dose back because her dose increase had coincided with poorer head control and increased coughing/retching/choking. It appeared that the baclofen was relaxing her neck/shoulders area too much, and in a potentially dangerous way, so that set the limit on how much it could help her arms and legs.

Hands/Feet- We focus Keira’s Botox injections primarily on her hands and feet. Unfortunately, she can only safely get these injections every 3 months. And the Botox is at its best for only about 1 month out of each 3. Still, it’s probably worth it. Keira’s toes get so gnarled that they overlap each other and make it difficult (sometimes impossible) to wear any kind of shoes. However, at it’s peak, the Botox has her toes looking nearly normal and for a couple weeks she can sport her cute sandals or boots.

Corwyn's Cause

They recently asked for our family's story and an account of what Corwyn's Cause means to us. I wanted to share it with you. Check them out at https://www.corwynscause.org/

We are the Halls! Jeff and I are both psychologists working primarily with children who have neurodevelopmental differences. We have 3 children. Liam is 13, Ronin is 10, and Keira is 6. Keira came to us after a seemingly typical pregnancy, but we soon learned that her brain development had been severely compromised by a congenital infection of cytomegalovirus (CMV). Keira cannot walk, talk, or hold up her own head. Her life has been complicated by many illnesses and surgeries. Most recently, we’ve discovered that she is growing deaf, so Keira is rocking some colorful hearing aids while I take a crash course in hearing loss, cochlear implants, and sign language.

Keira has a beautiful smile, an effervescent laugh, and more spunk than you’d imagine possible for a child with her limitations. Keira loves nothing more than being with her brothers, swimming, and drinking coffee. Black coffee. She has taught us so much about love and life. She helped inspire Idaho’s first legislation to further education about CMV, which is more prevalent, devastating, and preventable than most people know.

In spite of all the beauty we behold with Keira in our lives, it is also chronically stressful and difficult. The best way I can think of to describe our life is that it’s like having a newborn baby all the time. We are always watching her. As a family, we always need help. The needs of the older siblings often get pushed aside.

It is difficult to ask for help in the in-between times. We are lucky to have friends and family who are there for us in crisis, like when Keira is in the hospital. Corwyn’s Cause has made a welcome and measurable difference in our lives. Corwyn’s Cause has truly provided our family with things we didn’t know we needed. Monthly housekeeping frees up a lot of time and mental energy for me to do things with my family that would go undone otherwise, like swimming or an ice cream date with one of the boys. Corwyn’s Cause has provided us with gift cards for St. Luke’s, which pays for meals in the cafeteria when Keira is in the hospital or even a mocha to treat myself on busy appointment days. One of my favorite gifts from Corwyn’s Cause was the Christmas lights they put on our home last year! Christmas lights were something we’d always wanted to do, but it was so far down the list of priorities that it never got done. What a delight for us to see this festive display every time we came and went!

Corwyn’s Cause reminds us that there are other families out there who understand what we are going through, even in the relatively steady/”normal” times. And, through Corwyn’s Cause, we have access to those families. We can message through Corwyn’s Cause on facebook, to get moral support or to find a way to give our used equipment to someone who needs it, or to get advice about where to get the best size 7 diapers in the valley. Families who are associated with Corwyn’s Cause, I’d say, are families who are all dealing with the unique struggle and beauty of raising one or more children whose lives are very limited. We are families who don’t want to be alone, ostracized or forgotten. Neither do we want to be pitied, patronized, glorified, or smothered in platitudes. Corwyn’s Cause gets it because Corwyn’s family is one of us. Corwyn’s Cause meets us where we are. No obligations. No apologies. No strings attached. I know of no other charity of its kind.

Finally, Corwyn’s Cause hosts events for us to all come together. Events for the moms. Events for the dads. Events for the whole family that are inclusive of the typical siblings, too! Keira loved the summer event, where some BSU athletes RAN Keira, in her wheelchair, across the finish line of the race track they had set up. At the winter party, she loved getting to be so close to the live musicians that they could sing in her ear. What precious opportunities Corwyn’s Cause has provided. We can’t thank you enough.

                                                                                                                                       -Bekah Hall

Ears, Part 2

The results from Keira's hearing test were not what we had hoped. Her hearing in her left ear (the one with an aid already) has gone to severe loss, across the board. Her right ear now has loss, too; moderate at the lower pitches. That's a big change in just 3 months. We'll be going in tomorrow to get her fitted for a second hearing aid.

This stinks. It really does. But there is also a part of me that is relieved, like I always am, to receive a diagnosis. I like to know the plan. I like to know in what direction we're moving, and what course to take. To know we're pursuing the "right" path, provides me with some security, false or not.

Because Keira's hearing was worse, not better, they did not put tubes in her ears and we will not continue steroid treatment. Instead, I expect that we'll work with her hearing aids and work toward cochlear implants eventually. We'll try to make progress with the AAC devices (our second trial machine is sitting on the hearth right now, waiting to be put to use) and a person from IESDB (Idaho Education and Services for the Deaf and Blind) will start making home visits to teach us some sign language.

Dr. Park emailed me on Friday afternoon from Salt Lake to see how Keira's test had gone. When I told him the results he was surprised. He wondered if perhaps she has had a flare up of CMV and asked that she get tested to see. Having this task to do helps me cope. He is a leading specialist in this area, after all, and maybe Keira's fight will further his research and help other kids in the future. They don't really understand yet what it is about CMV that causes hearing loss and Dr. Park's interest in Keira's specific case makes me feel that maybe he's on the verge of a breakthrough.

Furthermore, after observing a couple of Idaho physicians seeing his recommendations and protocol through, Dr. Park directly asked us (me, the ENT, and the audiologist) whether we would be willing to help get our local hospitals on board with targeted screening.* He noted that he's been able to get hundreds of hospitals across the country on board with this and he sent me the published research articles he's used to support it. It's so exciting to see that what we've been through with Keira could get things moving in this way. Something like getting the ENT to talk to the hospital administration about CMV testing is not something that I could have done without Dr. Park's assistance. And it won't even require legislation! I'm pretty excited about that.

I love you all for reading and supporting us.

*Targeted screening refers to CMV testing that is done when a baby fails his/her newborn screening test. This is important because CMV is often the cause of childhood hearing loss, but if it is not tested and diagnosed within the first few weeks of life, we can never know for sure whether CMV was the culprit. Utah has passed legislation for targeted screening and other states are working on it. Targeted screening is different from universal screening, which would be if CMV were part of the newborn screening panel, in which case all newborns would be tested for CMV. No states, that I know of, do universal CMV testing. However, CMV has been officially nominated for inclusion on the U.S. Recommended Universal Screening Panel.