Tablet of her Heart

It’s been about a year since Sophie finally began using words to communicate her wants and needs. I can still remember so clearly those first few words she spoke in the early days of last fall. I remember every day the activities we did were made into speech therapy. I remember putting together puzzles and practicing SO hard for the simplest of sounds. And I remember success. I remember so clearly taking walks in the evening as a family, working so hard on her sounds.I remember the first time she uttered a two syllable word with a changing sound — “Micah.” So sweet. We practiced that night after night walking around the neighborhood with the kids in the stroller. It’s hard to wrap my mind around the reality that she spoke that word, “Micah,” last October, and now she’s speaking in full sentences! I am so proud of all the progress that Sophie has made in this last year. It is truly miraculous in my eyes, and I am so thankful that her journey with apraxia has been so different than I imagined. God has been good to my girl, once again.

I realize how quickly time is passing, how fast she is growing. And I want to remember these sweet days, the things she likes to tell me now that she has the words to do so. I wish I could capture these things in exactly the way that she says them, because it’s so precious. Like basket is ‘bakset,” and want is “nant” and some of her ee’s and oo’s slide into aa’s — like “I’m saa funnaay!” like an old Italian man. Pull ups are “pup-ups” and spaghetti is “skabetti,” Animals are “amisals” elephants are “ephanants.”
She loves to tell me “I don’t nant to!” and “I need hep!” or “PEEEEEEASE!” when she really wants something. Recently she’s also started saying “I nant to do dit all by maself” which is really actually sweet to hear after so many years of dependence.

When I ask her how school was, she tells me, “Good. I payed [played] at the sensaddy [sensory] table and the block adeeda [area].” Or, “I payed wiff ma fiends [my friends].” Or, “I ike Misses Pool.”

To her brother she says, “I nant a turn peease,” or yells, “MIIIIICAAAAH!” when she’s mad at him for stealing her toy. And in the good moments, she reminds him, “It’s ok. It’s ok. Jesus uvs you.”

She runs into the kitchen when I’m cooking and says, “Hey Mom, watcha makin for dinna?” And when I answer her, she responds, “It’s gonna be so nummaay! I’m gonna eat it all up!”

Most of all I love to hear, “I uv you so much Mom. I nant a hug and a kiss.”
These are my sweet memories of my four year old Sophie, growing so fast she’ll soon be five. I can hardly believe it. There will always be difficult days, days where the differences get the better of me, and the sadness creeps in. But when I step back and gain perspective, when I take it one day at a time, I know these days are precious and I treasure them in my heart.

We have two new speech therapists this fall, one at school, and one through our private agency. When I learned that we would be moving to new therapists, I wasn’t overjoyed, because transitions can be challenging and they can use up valuable time in which we could be breezing forward with someone that we don’t have to get used to. However, it has turned out to be more than fine. Sophie has been seen by new sets of eyes who never experienced “silent Sophie.” Our private therapist in particular finds it difficult to comprehend, I think, that Sophie was pretty much nonverbal one calendar year ago. Of course she’s read the initial evaluations and reports, the first sets of goals, the therapy notes. She sees the documentation in front of her, but when she looks at Sophie today, just a little over a year past her original apraxia diagnosis, she doesn’t really see apraxia anymore.

When I shared this with my mom, she asked, “Does apraxia usually resolve that quickly?” No. No it does not. Apraxia is a years long journey for most children who are diagnosed with it.

Because of our new therapist’s questions, I’ve reflected a lot on Sophie’s speech development over the past year. She definitely DID show some major indicators for apraxia last summer when she was diagnosed. She could not produce any intentional sounds. She certainly couldn’t sequence sounds. Her receptive language was leaps and bounds beyond her expressive language abilities. There’s no denying its presence at that time. But now, she really shows very few signs of apraxia anymore.

Children with apraxia struggle a great deal with sequencing sounds. They can repeat the syllables “po” “ta” and “to” but to say the word “potato” causes a great deal of difficulty. Sophie has no trouble with this word, or with magazine, restaurant, octopus, and many other multiple syllable words. Really the only remnant of apraxia that we can see is that she is still unable to plan certain motor movements, such as sticking her tongue out, clenching her jaw shut, making her lips move in more complicated ways (think, fish face), and moving her tongue up and down or side to side. Those are motor planning issues. And they do affect the clarity of Sophie’s speech to some degree.

According to our new therapist, Sophie’s current speech issues most closely resemble a condition called dysarthria, which is a speech disorder associated with brain injury, stroke, and more serious disabilities such as cerebral palsy, Huntington’s, Lou Gehrig’s disease, muscular dystrophy, MS, parkinson’s, etc. Fortunately, Sophie has had multiple MRIs, among other tests, and so we feel confident that she does not have any of these disorders, nor dysarthria itself. Her therapist also specified that she would not diagnose Sophie with this disorder, but that her issues resembled it. She actually feels that most of Sophie’s current issues are connected to her hypotonia (low muscle tone). There are 8 muscles in the tongue alone, and coordinating those muscles when they are low in tone will require extra effort, not to mention the other facial muscles, and muscles of the upper torso that work to form our speech. So we have a fresh take on Sophie’s speech problems and new ideas for how to help her progress.

I’ve been left wondering many things. Has Sophie’s muscle tone been the issue all along? Was she misdiagnosed? Or has her apraxia resolved at a miraculous rate? These are questions that we may never really be able to answer. And if you know me, you know that I’m not really too concerned about answers. I’m just so pleased that Sophie is doing so well with her speech. In my heart I just believe that whatever Sophie’s speech disorder was or is, God has been so good to her and to us in the lightening speed at which she has developed speech in this past year. We remain thankful and ever aware of his goodness to us.

While Sophie is on summer break from preschool, we’ve been working on potty training. I’m realizing it’s as much a training for me as it is for her. I think she’s going to be one of those kids who always needs accountability for a while. With the start of school closing in, I’ve been much one diligent in my efforts. So, we have this sticker chart, because Sophie loves sticker charts, and we needed a way to moderate the number of prizes she could get. Let’s just say, our past efforts at potty training have gotten too pricey. So, when she gets 10 stickers, she gets to go to the Dollar Tree and pick out a prize.

Today we reached the 10th sticker, and she was elated to go and get her prize. Some visits to the Dollar Tree take an eternity because she cannot decide which trinket she wants to bring home. Today’s visit was very prompt. We walked in, and she spotted a little plastic tea set and said, “This is it. Let’s go pay.” And so we did.

She didn’t want to wait in line and tried to force her way in to the belt with all of her 36 inches and 35 pounds of strength. Fortunately, the man ahead of us was a kind grandfatherly sort of fellow. He cheerfully said to my little line ditcher, “Hello, how are you? What’s that you’ve got there?”

Sophie answered (to my great surprise because she rarely dares to speak to people she doesn’t know well, out of anxiety, or because she is ignoring them), “It’s a tea set!”

He matched her enthusiasm, “A tea set?! That’s wonderful!”

“It’s wonderful!” She replied.

He then turned to me and asked, “How old is she? One and a half?”

One and a half? She’s 35 pounds! At first I really wanted to be upset. But, I have prepared for these moments and promised myself that I would be gracious towards others. So, I replied, “No, she’s four. She’s on the small side, I guess.”

Without missing a beat, he said, “Oh! Yes, well, I was going based off of her speech.”

Again, I had the opportunity to be upset and hurt. Because I know how much progress we’ve made in a year. But, I promised that I would be gracious, and now I had an opportunity.

“Well, she actually has a speech disorder. One year ago she was not able to say one single word. So she’s made a lot of progress this year, and we’re really proud of her.”

His response blew me away: “Oh! Well, praise the Lord!”

“Yes,” I agreed, “that’s what we do.”

“What’s her name?” He asked me as he prepared to leave the line for the door.

“Sophie,” I answered.

“Sophie, it was nice to meet you,” he said with a wave.

“Tell the nice man to have a good day,” I instructed Sophie.

“Have a good day!” She beamed.

God orchestrated an opportunity for me today, while I’m up to my eyeballs canning green beans, to go to the store and meet this man. Some people wonder why bad things happen, like a speech disorder, a disability, an illness. But I don’t wonder. I know. It’s for moments like this where I can share our story so that a perfect stranger has a chance to glorify God with us.

You can watch a video of Sophie enjoying her tea set which will always remind me of this beautiful encounter: sophie_tea_video2

Last week we visited our geneticist to follow up after Sophie’s first round of genetic testing. The testing for Weidmann-Steiner Syndrome returned normal with no defect on the particular gene for that disorder. This was great news! Weidmann-Steiner can have other potentially serious heart defects, so we are relieved not to need to consider that in the future.

However, that left us with no new direction to consider for Sophie. So, our geneticist decided to present Sophie to the entire team of genetics doctors at the hospital to see if any of them had any ideas. This week I received a call that someone on the team suggested Coffin-Siris Syndrome, and our geneticist would like to test for it. This syndrome involves mutation of a different gene than Weidmann-Steiner (we have 23,000 genes in our bodies, making this search literally like a needle in a haystack). Like any other syndrome, it can have a profound affect or a mild affect on the child’s health and development. In addition to differing physical features (some of which Sophie may have and some she does not), speech delays, mild to profound intellectual disability, and low muscle tone, it can also indicate kidney problems. That fact alone makes it worth testing for.

We’re not on some hell-bent quest for answers. We’re not looking for a named syndrome to suddenly make it all better. But we are trying to be wise about Sophie’s future. If there is a genetic mutation, it is possible that internal organ systems can be affected, and that is something to be aware of and prepared for. So our search will continue. God bless BCMH for their financial assistance.

I confess that conversations like these with special doctors always make my heart bleed a little bit. I don’t like the scrutiny that my beautiful girl comes under (even with doctors who have the best of intentions) for her physical features, which I feel are just perfect. It’s hard to consider the possibilities that the future holds. It’s hard to talk about the areas where she is lacking. It feels unfair… because we are all lacking in some way… so why are her ways so important to look at under the microscope? I tell myself all the time when I catch myself thinking I just want a “normal” life for Sophie, what IS normal anyways? Which one of us is perfect without anything “abnormal” in our lives? I tell myself, she will grow up just fine. I want to believe that. With all my heart I do. But sometimes heart is just not enough and doubts creep in.

But faith…  faith is enough. And I hold on fast to these words: “‘For I know the plans I have for you’ declares the Lord, ‘plans to prosper you and not to harm you, plans to give you a hope and a future.'” Andrew and I chose this verse as the text for the sermon at our wedding, along with another well-known passage: “Trust in the Lord with all your heart and lean not on your own understanding; in all your ways acknowledge him, and he will make your paths straight.” So sweet how these verses have stood with us through so many trials over the 14 years that we’ve been together, a constant reminder that God is directing our steps with all goodness and love. With every difficulty we encounter, he meets us with grace, strength, and truth to carry us through.

We really appreciate continued prayers for wisdom on the part of Sophie’s doctors and for her continued growth and development. If anyone feels led to pray about potty training, I will share my mansion with you when we get to heaven.

There’s something overwhelming in looking to a future that is dark with uncertainty and question marks. We move through life with such an unrealistic sense of certainty in what’s next. We have a plan. We imagine what the next days, months, years may hold. We think we know. But there are moments, times, events in our lives where we realize with keen certainty that we do not know. Truly we don’t ever know what is coming down our path. But we think we know.

When Andrew and I got married almost 9 years ago, we thought we knew. We had a plan. When Sophie was born, we thought we knew. The future, ever uncertain, was envisioned with certainty. But when Sophie was four months old, she was diagnosed with an unspecified developmental delay, and that future we saw with such clarity was darkened into obscurity. Her future was uncertain, literally.

And God asked me to trust him. To place her in his hands. To put her future, our future, into his loving care. To accept, to embrace his plan. Four years later this month of June, I can say, he is fully worthy of our trust. He has got it. If we step forward into that uncertain, obscured place, we will find abundant blessing. In this journey of four years, I have found him to be entirely trustworthy. Entirely.

There’s a story that I love of one of the kings of Judah, Jehoshaphat, found in 2 Chronicles 20. Jehoshaphat learns that a vast and powerful army is preparing to attack his land. When he hears the news, the story says, “Alarmed, Jehoshaphat resolved to inquire of the Lord.” Jehoshaphat was surely alarmed to hear this news. We’ve all felt that moment of panic rising up in our chest, or that sinking feeling, the sting of tears in our eyes, the fear in our hearts, anxiety threatening to take over. That’s alarm. But, the very next action listed in Jehoshaphat’s story is “resolved” — “Jehoshaphat resolved to inquire of the Lord.” To resolve is to make a level headed, firm, unwavering decision. For me, it’s not usually the action that follows alarm. Usually alarm is followed by all those things I described above — fear, anxiety, tears, maybe anger, etc. But not Jehoshaphat. He feels the weight of the bad news, he feels the alarm, and then he chooses to bring the matter before the Lord.

One of the first things that Jehoshaphat does in his prayer to the Lord is to remind himself of who God is. He reminds himself that he serves the God who rules in heaven, who has all power and might, whom no one can stand against, who has aided his people at every turn in history and performed many miracles. I love that Jehoshaphat takes these moments to focus on who God is, because I think that this choice restores perspective.

We are so quick to turn our eyes to our circumstances, but then our circumstances feel bigger and bigger. What we need to do is turn our eyes to God and find that he will be bigger and bigger, while our circumstances will grow smaller. Perspective is a valuable thing. In focusing on God, we can restore our lost perspective when the question marks come.

The final statements of Jehoshaphat’s prayer mirror my own decision four years ago when we learned that Sophie could potentially have serious medical problems: “We will stand in your presence … and cry out to you in our distress… We do not know what to do, but our eyes are upon you.” Jehoshaphat chooses a position of standing, his confidence evident in his choice. He is not undone and weakened by the bad news he has just received. I wish I could say I never crumpled under the weight of our bad news, but that would be a lie. I know that I did. But, after I laid there awhile in my “alarm,” I finally found the courage to say “I do not know what to do, Lord, but my eyes are on you.” It came down to a choice really: fear or faith. Fear the question marks, or have faith that he had a good plan. My emotions wanted to run wild in those early days of Sophie’s diagnosis. But my mind and my soul resolved to trust the Lord. And so I made my choice. And I have never regretted it.

I cannot even begin to list the ways that we’ve been blessed on our journey with Sophie. Yes, there has been fear, anger, resistance, certainly. Acceptance has been a process. It always is. But what I want you to hear most in Sophie’s story right now, four years later, is that he is trustworthy. No matter how many question marks, how darkened your vision, how sharp the bend in the path of life, I say this: he is certainly trustworthy. He is more than able. If you resolve to trust him, to stand before him and cry out for help, to fix your eyes on him, he will be faithful and good. You will see it along the journey, and you will see it in breathtaking clarity as you look back from years down the road.

Choose faith. Move forward in faith. There’s nothing dark about the plan from his vantage point. It is the best plan. Believe it. Choose it. Walk in it, resolved, not alarmed.

**I’m including a link to a song that still brings me to tears, that helped me resolve to trust some four years back. Have a listen: Nichole Nordeman — “Someday” https://www.youtube.com/watch?v=bVx9FUQvpXw

I’ve had quite a few people ask me lately how Sophie is doing at preschool, if she will go to kindergarten on time, if she will attend a regular school, etc, so I thought an update on her progress might be helpful to those who love Sophie and pray for her faithfully. We so appreciate each of you and we never underestimate the power that your prayers have had and continue to have in Sophie’s life.

Sophie’s first day of preschool, 2015

When Sophie began the school year in fall of 2015, about 9 months ago, she was just beginning to make some sounds and say very simple words (ma, up, go, ice, bye). She used many, many signs, and so we had some indication that she understood quite a bit, but we were unsure before she began to verbalize where exactly her cognitive abilities lie. As the year has unfolded and her verbal skills have progressed, we have been delighted in discovering the wealth of knowledge that was trapped inside her head.

• We discovered that Sophie can count to 20.
• We learned that Sophie can recognize almost all of the letters of the alphabet, most of the time, in any order presented.
• We found that Sophie knows every animal, even the weird ones, and all the noises that they make.
• We thought before but had confirmed for us that she knew all of her colors and shapes, as well.
• Most recently, in constructing Teacher Appreciation cards and Mother’s Day cards, we have learned that Sophie knows how to spell her name and can write it with hand-over-hand assistance.
• Sophie knows a lot of songs, though she cannot truly sing them yet.

So, having just turned four years old, Sophie has met some of her kindergarten benchmarks already! She has another year of preschool to complete before she would even be eligible for kindergarten, so I have little doubt in my mind that she will be ready to go when she’s five (although we will still have the option to do an additional year of preschool if we want to give her more time). I can hardly conceive of it. A year ago she was not speaking at all. In just 8 months, she went from the simplest of words to speaking in short sentences. It is truly astounding! In addition, at a mid-year developmental assessment, Sophie scored just below average for her age range, rather than being categorized as developmentally delayed, which she has been since four months of age. We are overjoyed to have finally shed that label and are continuing to work towards developmentally average and beyond!

Sophie will still require speech therapy for quite some time yet. Her apraxia is still at play in the extra difficulty that she has in formulating certain sounds which she should be able to form and in using the correct sound in the correct place. She’s got tons of sounds now, but her mind struggles to plan the right sound at the right time, which means that her speech is often difficult to understand. Our current goal with speech therapy is to increase her precision with vowel sounds so that she is more intelligible to those she speaks with. We probably understand about 80-90% of what she says. I imagine that outsiders would probably get about 25-30%.

Sophie spent three and a half of her four years and three months in silence, so it’s not at all odd to me that she is still learning about what to say when or how to act in certain social settings. She’s making a lot of progress there, beginning to learn what to say when, and I hope that as her speech improves and she learns social cues for speech, her social awareness will increase. In spite of her social differences, Sophie experienced beautiful acceptance a classroom full of sweet friends and two BFFs (pictured: Evelyn & Collins) on top of that. God truly blessed Sophie with caring friends and nurturing teachers. Even another child in her class who routinely struggled with behavioral issues consistently showed compassion to Sophie. Her teacher told me at our spring conference that Sophie was by far the most popular child in her class! This is totally amazing to me! Not because I don’t find Sophie wonderful, but because as adults, we are so focused on interacting with those who have something to give us in return. Seeing Sophie interact with her friends at preschool reminds me that friendship is not about what I can get out of it or what someone else can give me. It’s just about giving to others that you enjoy being with.

On the medical front, Sophie’s genetic testing for Weidmann-Steiner Syndrome has just been completed. Her test revealed that particular gene was normal! I was overjoyed to hear these results! So many happy tears! Thank you, Jesus! We return to the geneticist in July to see what our next steps may be. This test was run on just one gene, so choosing another gene to look at is, as you may imagine, like looking for a needle in a haystack. We may be at a dead end for now, which is totally fine because Sophie is doing so well. We don’t NEED to find an answer because she’s not deteriorating or failing to progress. So that’s very GOOD. It’s likely that somewhere in one of her many genes there is a minor deletion, duplication, or sequencing error, but this occurs in many individuals. It would account for the differences we have experienced thus far in Sophie’s development, but it may not be a named syndrome, and we may never know which particular portion of a certain gene is affected. As long as she’s doing well, it’s really NOT a big deal to know the specific gene and if it is a named syndrome.

Last day of preschool

As I reflect on this school year, I’m simply overwhelmed by how much progress Sophie has made. Every small step feels miraculous and wonderful! I am so thankful that in a year full of other kinds of difficulty for myself personally, God has encouraged my heart so much through Sophie’s progress. He has been faithful time and again. I know that he is enabling her awesome progress and blessing her with favor in addition. He has been so very good to our sweet Sophie girl! And I know he will continue to be. To think that when she was four months old, we had to question if she would live to see four years old… Now here we are with a developmentally “slightly below average” chatter box, romping around, loving life. So very good. Thanks, God.