Tuesday, November 12, 2019

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! 

Monday, November 4, 2019

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.



Friday, October 11, 2019

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.







Tuesday, March 5, 2019

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





Monday, February 18, 2019

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.


Wednesday, February 13, 2019

Ears

CMV is a leading causes of childhood hearing loss. It's a leading cause of a lot of problems, actually, but ENTs and audiologists have their finger on the pulse of CMV whereas other specialties don’t. One of the few official standards to follow when it comes to medical supervision and care of kids born with congenital CMV is to monitor their hearing. Kids born with CMV often fail their newborn hearing tests. Some pass their newborn test but lose their hearing later. Many suffer progressive hearing loss.

Starting from birth, Keira has had her hearing tested every 6 months. She's had tubes placed a couple of times; not because of ear infections, but just to keep them clear of fluid and pressure so we could get adequate hearing test results. Keira has actually had very good hearing. In fact, I found it somewhat annoying that so much focus in CMV research and literature was in regards to hearing. Hearing, I felt, was the least of my worries. When you've got a child who can't see well and can't speak and can't walk and can't eat, hearing loss doesn't seem like the most urgent thing. But the truth is, many kids born with CMV have hearing loss and no other unfortunate disabilities, so hearing is of utmost importance to them. I get that.

In October, Keira had her first sedated ABR hearing test. This was a bittersweet milestone. Until then, Keira could have all of her hearing tests done while she was awake (by putting probes on her head and reading the brain responsiveness to various sounds). However, she has recently gotten too wiggly for a non-sedated test. The increased movement is awesome, though the flip side of that is that now she has to go under general anesthesia for her hearing tests.

The results of her first sedated test were bad! Her right ear tested normal, but her left ear tested as moderate to severe hearing loss. That was a huge decrease in under a year's time. It was a pretty big blow. Once again, when things felt somewhat steady and predictable for Keira and for us, we were reminded of the precarious nature of her health, her fragility, and of course the idea that she could rapidly decompensate and leave us for good. 

I wasn't altogether surprised about the hearing loss, but the audiologist surely was. The audiologist thought that we were pretty much out of the woods, believing that the longer Keira went without hearing loss, the less likely she was to ever have that happen. I wasn't so confident. One think I knew best about CMV was that it causes progressive hearing loss in children. Plus, I'd heard Dr. Park's recent updates regarding his vancyclovir studies.

When Keira was born, she took vancyclovir, a dangerous antiviral medication, for 6 months because Dr. Park had shown that this reduced the incidence of progressive hearing loss for children born with CMV. It was (and still is) the only standard recommendation for treatment of babies born with CMV. Keira took the medication with no problems, took her routine hearing tests, and worrying about hearing loss was put on the back burner.

In September, I attended the national CMV conference in Vermont. I listened to Dr. Park speak about his updated research. They were beginning to find, unfortunately, that the vancyclovir was not so much preventing hearing loss as it was delaying it. The children in his study were still losing their hearing, but it was happening later. This is unfortunate, but the vancyclovir is still beneficial because it is of huge importance for kids to have their hearing early in life to help understand language and communication.

So I thought that his lecture was interesting and within weeks after returning from Vermont, we got this negative result from Keira's ABR. In response, I shot Dr. Park and email. I basically said, "You know that thing you were talking about? Yeah, we just saw that happen with our daughter." Dr. Park responded personally and informed me that he was doing some new research on a treatment method that was showing some modest results and he'd be happy to talk to us about it if we were interested. Jeff and I made plans to go and meet with him at Primary Children's in Salt Lake over Christmas.

In the meantime, I went into high gear, trying to do anything and everything I could think of to help Keira in the event that she went completely deaf, like, tomorrow. I got super critical of her current speech and language therapy and decided they were moving too slowly. I got her started with a 2nd, additional speech therapist to do some supplemental work with Keira. I got her on the waitlist to see a 3rd, one who is renowned across the valley for expertise in AAC (alternative and adaptive communication). We got in contact with the Tobii Dynavox company, one of just two that makes AAC devices with eye gaze tracking. We initiated a 4-week trial of a "Tobii" device. "Just to see."

After Christmas, we left the boys with their aunt Shelly and cousin Sam and took Keira Salt Lake City. We met with Dr. Park, which was quite satisfying. As someone who knows CMV, he agreed that it’s likely Keira could lose the rest of her hearing in both ears and quickly. Bad news, yes, but so encouraging to have my suspicions validated. I felt like the doctors in Idaho thought I was overreacting. Park told us about a study he is doing that was just, I mean JUST, proving to have some measurable results. After following his protocol, some kids were not only having their hearing stabilized, but actually improved! 

The protocol is this: As soon as we got home, we put Keira on a 3 week regimen of oral (G-tube) steroid (prednisone). Then, after a few weeks we’ll have her hearing tested again and if there is ANY improvement, they will put tubes in her ears and we’ll give her steroid ear drops indefinitely. He also recommended testing her hearing every 3 months. 

Keira survived the oral steroid. Park told us that most kids take that dose without issue. I didn’t tell him that Keira’s mother once had to discontinue a steroid eye drop, an eye drop!, because it was making her crazy. During the first week especially, Keira was cranky, flushed, and sometimes inconsolable. But it got better as we tapered her down. Plus we got some interesting results right away. 

We had to coordinate the care protocol with a local ENT. While at that appointment, the pressure in Keira’s ears tested normal! Normal! That hadn’t happened in years, if ever, and is most certainly due to the steroid. This gives us hope that she will test well enough to get tubes and ongoing intervention. 

In the meantime, Keira received her very first hearing aid. It’s been unremarkable. She tolerates it swimmingly, but we don’t really notice any difference in her hearing or her behavior. And we did her 4 week trial of the Tobii Dynavox. 

Keira's response to the Tobii was really incredible. It broke my heart and made it soar all at the same time. 
To train her to use the device, first we have to teach her how it works. It came equipped with several games for that purpose. For example, on “Pie Face,” there is a small picture of one of her family members in an otherwise black screen. When she looks directly at the picture, it triggers animation of a pie in that person’s face then switches to a new photo in a different portion of the screen. 

The Tobii is basically a glorified iPad that has a strip across the bottom that tracks her eye movements. When setting it up and calibrating it, you can see where exactly she’s looking, which was fascinating. We saw that when trying to purposefully look for something, she was really only using one eye, her right eye. Then we observed that she had a hard time looking at things on the left side of the screen. To compensate, she would use all of her strength and rapidly move her head to the left, trying to capture the picture with her right eye. After 10 minutes of this, her face will be flushed with the effort. But, oh, was she ever proud of herself! She was controlling something in a way she never had before. And in spite of her visual impairments, she was getting it!

Eventually, the idea is that Keira would graduate from the games to communication boards. With her eyes, she would choose pictures off a grid to represent what she wanted and the Tobii would “speak.” We’ve got a long way to go with that. Just like any computer, the Tobii has its glitches and limitations. The programs available for young children are few and with the eye-gaze adaptability even fewer. What I had anticipated was an experience like we’d had with the boys on the iPad. I thought we’d be able to choose from a variety of apps featuring cute, colorful characters teaching the alphabet and manners and rhyming. I was sorely mistaken but found instead that I can create these programs myself. (Insert picture of my head exploding). Great. I’ve always wanted to dabble in computer programming and curriculum building. Not. 

I practically kicked my heels when I was able to finally put that damned machine back in the mail. It felt like so much pressure. It’s much more cumbersome, too, than just asking Keira which thing she wants and waiting for a response in her expression. But, she may not be able to hear us always, so we’ll keep on keepin on. In fact, we expect a delivery today of the PRC, the other option in AAC eye-tracking equipment, to begin another trial. 






So all this brings me today. As I write, Keira is getting sedated for her ABR. If the results are what we hope, she’ll have tubes put in her ears. While we’re at it, she’s also going to have Botox injections, xrays, and dental work. 

If K’s hearing has not improved, then we'll just deal with what comes as far as her hearing is concerned. The worst case scenario is more hearing loss, another hearing aid, and eventual cochlear implants. 

She was as sweet as can be this morning. She gets nervous at the point that we go into the pre-op room, but she understands what we are telling her enough that we can visually see her trying to stay calm. She chose peach chapstick for her mask this time and she has Lovey and Peppa along for the ride. 




Friday, February 1, 2019

Turning 6





Just like that, another year has gone by and, as is the case with all kids, year 6 brought some interesting changes.

First and sadly, Keira graduated from (read: "aged out of") the intensive therapy program that we fought so hard for her to be a part of. I've probably mentioned it before, but it's a program that Keira attended 3 days per week, for 3 hours each day. It included PT, OT, speech, and feeding, plus lots of social interaction with kids much like her.

The program is open to kids ages 3-6 who are in need of all the services I listed. Though I fought long and hard from the time Keira was 2, she wasn't actually accepted into the program until she was about 4 1/2. The excuse I got was that they didn't know if she had the stamina to endure 3 full hours of therapy at a time. I didn't know how they could be so confident that that was the case and I was frustrated when child after child, often not even 3 years old yet and/or with higher functioning entered the program ahead of her. Note that this is the only program like this in the area and it can only accommodate 12-15 kids at a time. Keira finally got into the program when we agreed to have a one week "trial" to see how she would hold up. She blew them away [gasp!] not only with how she "held up," but how hard she worked and how much she enjoyed the program.

So, at age 6, the program lets kids go and helps support the child's transition into public school. Keira attended preschool last year at the same place where the "medically fragile" kids from our town go to elementary school. As such, I was pretty familiar with the school program and personnel and felt pretty good about her spending more time at school. For one, I perceived, we would spend a heck of a lot less time in the car, driving her back and forth to appointments every day. And Keira would be able to continue seeing all of her therapists, just less intensely. Just an hour per week for each of them.
Keira and Grandpa at the tea party

Still, I was sad for the program to be ending. Who else in Keira's life, I wondered, would ever provide her with the experiences that this place did? While in the program, Keira played with dolls, went camping in a tent in the middle of the group room, had a tea party, was in a play (complete with speaking parts, sets, and costumes), went on an Easter egg hunt, and played baseball. I was stupefied by how they managed any of these things.

Once early on when I picked Keira up, her therapist told me how much she had enjoyed playing with dolls that day. "How in the world?" I asked, because, naturally, my first thought is that I should try to duplicate this at home. Well. Picture this. One therapist to hold Keira in a sitting position and support her head. A second therapist to manipulate Keira's arms. And a third person to move and manage the doll, bottle, and other play materials for her. I was so happy for Keira to get to experience this "typical" childhood activity, but I also realized, and mourned the fact, that these types of activities could not be duplicated in "real life." So when the program's team asked me about how I was feeling about Keira graduating, I was nearly in tears when I said, "Who else is ever going to do these things for her in her life? Ever? I mean, how will she ever play baseball?"




As each child graduates from the program, they have a special ceremony that is unique to the particular child. This was the cutest. thing. ever. Jeff and I, and Grandma and Grandpa sat in the chairs on one side of the group room while each member of the group gathered on the other side with their personal therapist. Keira was rolled to the front by her therapist and her smile grew as she noticed all the familiar faces watching her. The therapist held her green pre-recorded speech button next to Keira's head and with one head nod and one phrase at a time, Keira said something to the effect of, "Welcome to my graduation! Lately, my favorite thing in the program has been playing baseball! So, today, we are going to play baseball. But first, we are going to sing! Repeat after me!"

She began "Take me out to the ball game..." Which we all repeated in song. Keira bumped the button with her head for each phrase in turn, something that they had been working on very recently. And she mostly got the turn-taking idea, but sometimes went ahead with a solo rather than waiting for us to repeat her.

"Now it's time to play." She reported which kids were going to be on which team....

"My team is up to bat first. Who gets to go first?"...

"I do!"

Keira's therapist helped her to hold the plastic bat in her hands. Everyone in unison shouted "One, two, three," at which point Keira was helped to knock the large bouncy ball off of the tee. Keira's therapist pushed her through all the bases while the crowd cheered. The smile on her face was to die for.

Each kid participated in their own measure. Some in wheelchairs. One kid was in a walker/gait trainer. One was lying prone on a skateboard-like contraption, propelling with his his arms. A couple were fully capable of walking, but needed lots of assistance to follow the instructions of the game. The "outfield's" job was to pick up the ball and put it back on the tee. Everybody was a winner.

It was just adorable to see how each kid participated in their own way and with their therapist's assistance. Each kid looked so proud and so engaged. It was a beautiful thing. I wished I could share that moment with the whole world.



After the game, the kids lined up and Keira was guided down the line and, in trick-or-treat fashion, each kid presented her with a laminated page that included his/her own photo, message, and artwork which was then compiled into a very special "goodbye" book just for her.

Soon after that sweet day, Keira started Kindergarten. I was so pleased that, without me even suggesting it, the IEP (special education) team decided that Keira should spend the mornings with an assistant in the general education classroom since she thrives on social interaction. I loved this idea, since most of the other medically fragile kids are unlikely to speak with or interact with Keira much at all. Keira is happy to go to school. Happy to ride the bus. Happy to come back home. And that's all I want. Learning is a bonus.

Here is a picture of Keira when we went shopping for her to get a new backpack and lunchbox for school. As usual, she's cracking up at Liam.


















This summer, I discovered that Keira LOVES strawberries.


So for her little party we had a strawberry theme, ate homemade strawberry ice cream, and she wore her strawberry pants. We were happy that Papa felt well enough to join us. Papa is one of Keira's favorite people (and I think the reverse may be true as well) and unfortunately for Papa, the two had a lot in common what with their eating problems and hearing aids. Keira's favorite gift this year was probably her "baseball," bat, and tee.
 

Isn't this the sweetest photo? I'd all but given up on school photography, but I feel like somehow, this photographer captured her. Kindergarten!