Made in His Image, part 1

I recently had the privilege of speaking at a women's conference under the title of "Made in His Image: Understanding Disabilities from a Biblical Perspective." Seems an odd topic to speak on at a conference for women, doesn't it? But there you have it -- that's what I was asked to present and so that's what I did.

I've spent a lot of time gathering my thoughts and putting them on paper (or screen) and thought I might post the transcript here for those who weren't able to attend the conference and who care what I have to say. There's a lot more that I wanted to say that didn't make it in because I was limited to an hour of speaking time and didn't think the ladies would appreciate my pulling an all-nighter on them.

I won't share the entire transcript in one post because it's too long and no one likes to read long posts (the presentation was about 55 minutes long). So, stay tuned for more over the next few days. I invite your thoughts and comments as I go along.

Made in His Image: Understanding Disabilities from a Biblical Perspective

Introduction

I’d like to thank my friend for asking me to share with you today. I appreciate the opportunity the Lord has given me to share some things on my heart with you. I want to spend our time together telling you a little about myself and my family and the path through life the Lord has placed us on, and wrestling through some tough God questions that we’ve encountered along our journey.

I grew up in a Christian home and the Lord graciously saved me from my sins at an early age. My parents are people who love the Lord and who are committed to His Word. There were many times when they would ask me, “Have you prayed about it?” and “What does the Bible say about it?”

After I graduated from college, almost fifteen years ago, I began to work with Answers in Genesis. Ten years ago, I met and, six months later, married my husband Seth.

We struggled with infertility for several years before I became pregnant with our first baby. Like most parents, we were thrilled and began dreaming about our baby’s future. When our son was born in 2008, Seth was sure he would be a star running back in the NFL and I knew that we had a future valedictorian on our hands.

And then the doctor visits began.

From the beginning of his life, Buddy struggled to eat, to sleep, to gain weight. So we were back and forth to our pediatrician’s office many times during those first few months. When he was two months old, our pediatrician heard a heart murmur in Buddy and sent us to the local children’s hospital where the pediatric cardiologist diagnosed him with a condition called supravalvular aortic stenosis. That means that there’s something wrong with a section of his aorta.

Let me stop here and talk a little about the aorta. Now, I’m not a doctor, and I don’t play one on TV, but I do want to share with you some amazing features about the aorta.

Photo courtesy of iradonline.com

After your heart pumps your blood to your lungs to pick up the oxygen your body needs, it brings it back through the heart and sends it out to the rest of your body through the aorta.

The aorta is the largest artery in your body. It carries and distributes oxygen rich blood to all the rest of your smaller arteries throughout your body.

The walls of the aorta consist of three layers of connective tissue and elastic fibers. These fibers allow the aorta to stretch to prevent over-expansion due to the pressure that is exerted on the walls by blood flow.

There’s a valve that allows the blood to enter the aorta and keeps it from flowing back into the heart. Isn’t that amazing? Doesn’t the way this aorta is structured – its ability to stretch and contract—just scream “there is a Designer!”? When we look at this, it’s easy for us, as Christians and those who believe God is the creator, to say, “Yes, of course, there’s a good, loving God who designed it all!”

But what happens to God’s goodness when a baby, our son, is born with an aorta that is constricted? Supravalvular aortic stenosis. Supravalvular meaning “just above the valve” and stenosis, meaning “constricted.”

You can imagine what happens when a section of your aorta becomes too constricted. Many babies with this condition undergo open heart surgery to correct it within the first few months of their lives. And some need multiple surgeries. Just since the beginning of this year, I’ve known about a dozen children who have had this operation. And there are many others waiting to find out when they’ll need to have theirs repaired. So far, Buddy hasn’t needed the surgery but we don’t know what will happen in the future.

In response to this, one lady said, “This is a problem I have wondered about—how physically imperfect newborns can be admired as "the handiwork of God," because it casts such doubt on God.”

She has a point. We routinely point to God’s handiwork in our children—they are fearfully and wonderfully made by the Creator, right? So do “imperfections” really cast doubt on the Creator?

Part 1, Part 2, Part 3, Part 4, Part 5, Part 6, Part 7, Part 8, Part 9, Part 10, Part 11

EEG results

We visited with the neurologists at our children's hospital to find out the results on the EEG Buddy had last month.

The EEG didn't show any seizure activity, but according to the neurologist, they usually have to induce a seizure through hypoxia caused by hyperventilation -- something they don't do with children as young as Buddy. So, she wasn't surprised that the EEG was negative. However, based on my descriptions of what I've seen Buddy doing (blanking out, not responding for short periods of time), she said he was definitely experiencing absence seizures and that, even though I was only seeing a few episodes per week, he could be experiencing anywhere from 2-100 seizures each day.

She also said, in answer to my question, that while some seizure activity is associated with the hypercalcemia those with Williams syndrome can have, absence seizures are not necessarily associated with WS. So this is something in addition to WS that we're now dealing with. (As far as we know, Kieran's calcium levels are in the normal range.)

So, for the course of treatment, the doctor prescribed one of three anti-seizure medications: ethosuximide, valproate, or lamotrigine. Getting him on a daily dose of one of these medicines (the recommended is valproate) was a no-brainer for both neurologists we talked to. It helps to control the seizure activity which would otherwise interfere with his overall development (imagine the problems you would have concentrating and learning if your brain were seizing up 200 times each day).

For us, however, the decision will take a bit more time. The side effects, of course, of the medicines are a concern for us. So, we'll be looking into each of them a bit more, asking the Lord for wisdom, and then making a decision based on what we think will be best for Buddy.

EEG Day

Last month, we went to our pediatrician to get a referral to the neurology clinic at the children's hospital for an EEG because I suspect Kieran may be having absence seizures. (Wow, that sentence has a lot of prepositions.)

Today was that EEG. I've been talking to Kieran about what would happen, hoping to prepare him so that he wouldn't freak out when the technicians started putting the electrodes on his head.

"We're going to the doctor in a few days and they'll put some things in your hair. But it won't hurt. It will be fun! And you'll get to watch videos with daddy! It will be fun!"

He seemed to believe me. When we got to the hospital, he told everyone who would listen, "It doesn't hurt."

And then, when we got to the neurology area, he told the lady, "It doesn't hurt." And she said, "You're right! It doesn't hurt! We'll just put little stickers on your head." Which I thought was a great way to put it, so I started telling him, "Ooh stickers! It will be fun to have stickers on your head! Stickers don't hurt! Yay for stickers on your head!"

And then we got to the EEG room. And the lady explained what they would do. And then, as soon as they laid him on the table so they could start stickering him up, he began cry uncontrollably. Before they started touching him.

And he continued to cry throughout the stickering process. But once Seth was able to hold him and he was able to watch Elmo's Potty Time on the TV, he calmed down for the most part, with a few occasional bursts of crying just to remind us that he didn't like having to do this.

 

And we all made it through the dreaded EEG.

 

We kept him up two hours past his bedtime last night and got him up earlier this morning so that he would be tired for the EEG (I don't think it worked), so he was pretty tuckered out on the way home.

 

We'll find out the results in a few weeks.

Kidney follow-up

Because of the looming snowstorm, we spent last night at SIL's place. They live about 8 miles (compared to our 40+ miles) from the children's hospital and Buddy had an early morning appointment there. This one was a follow-up with the urologist to check on his kidneys. By the sounds of his crying during the ultrasound, you would have thought the tech was dissecting his kidneys rather than simply taking pictures of them. Buddy kept signing "all done" throughout the 15-minute ordeal and I had to keep telling him, "No, not yet."
After it was over and the doctor had looked at the results, he happily told us that his kidneys looked fine, looked like they were growing well, and that he didn't need to see us again for a year. And there was great rejoicing.

Kidneys

Did you know that your kidneys can reflux? And that there's a scale measuring the reflux? And that having kidney reflux in the 4 or 5 range usually requires surgery? And that kidney reflux is detected by inserting a catheter in you-know-where, filling the bladder with liquid, and watching the kidneys do their thing on an X-ray?

Well, they do; there is; it does; and it is. And we found out all about it today.

You may (or may not) remember that early last year, Buddy had 2 UTIs (urinary tract infections). Standard operating procedure after a UTI is to send the child to our nearby children's hospital to have the kidneys checked out. And so there we went. His kidneys were poked and prodded and it was determined that, on the kidney reflux scale, his kidney reflux was a 3.

Today, we had a follow-up kidney ultrasound and cistogram (the thing with the cath) to find out if the reflux had progressed or abated. Two screaming fits (one during the ultrasound, one during the cistogram, oh, and one during the taking of the blood pressure . . . so make that three screaming fits) and four hours later, we were informed that his kidneys look good and his reflux appears to be in the same range as before, meaning no surgery is required at this point. We'll return for yet another visit to the urologist and his ilk in six months to again assess Buddy's kidneys. Who knew kidneys could be so exciting? And so, um, draining?

Another day, another doctor appointment

Our nearby children's hospital has 47 separate divisions or clinics (cardiology, genetics, radiology, etc.). We have had appointments with 12 of those 47 clinics. I hope it doesn't sound like I'm complaining because I'm not (at least, I don't think I am). I'm merely stating a fact--we've made many visits to the hospital these past 18 months. Almost monthly hospital visits to a variety of specialists simply weren't on the agenda two years ago when we found out I was pregnant with Buddy.

But, as usually happens, you just "do the next thing" (to borrow a phrase from favorite author Elisabeth Elliot), God gives grace, and life goes on. Today's visit was with the radiology division to conduct a swallow study on Buddy--to make sure he was swallowing food and liquids as he should be (a common WS symptom is trouble with swallowing). The outcome was that all looked good and he was swallowing as he ought. And so, we move on to the next doctor visit, ENT (otolaryngology) . . . .

Dentist

Williams Syndrome presents itself in (among other things) a variety of dental problems, including defective tooth enamel, which can lead (obviously) to tooth decay. Don't ask me to explain why the tooth enamel is defective because I can't--just accept that it is what it is (I'm so zen).

At our last appointment with the geneticist, he suggested that we begin monitoring Buddy's teeth now. Thusly and henceforth, I made a dentist appointment for him, which was today. At the appointment, we were given a questionaire to fill out. One of the questions was, "Do you think your child will react negatively to a dental exam?" I answered, "Um, yeah." Not really. I just checked the "Yes" box and moved on to the next question. Are there any 17-month-old children who do not react negatively to dental exams?

And, during the exam (which was all of 60 seconds and involved no instruments or high-pitched noises), Buddy reacted as I had predicted. The dentist had him lay down backwards on my lap and proceeded to examine his mouth while he screamed bloody murder. I suppose it was rather convenient for the dentist, since B's mouth was wide open.

The end result was that Buddy's teeth look fine--the enamel seems to be normal and without defect for now. We'll see the dentist on a six-month schedule to keep tabs on the teeth. In the meantime, I suppose I should remember to brush his teeth more routinely . . . .

Another echo

Yesterday, Buddy and I went to the hospital for another echocardiogram. The cardiologist said the results were positive -- the supravalvular aortic stenosis (SVAS) looked like it had actually progressed back into the mild range (it was a "30" (the upper end of mild) and this time is was "25"). His blood pressure was in the normal range, and all other factors that they measured looked good.

We'll have another echo in a year (instead of every 6 months as we've been doing). According to the cardiologist, if the SVAS continues to stay in the mild range, he won't need surgery on it, and we won't need to be concerned about it (as far as activity restrictions, etc.). And if, over the next few years, the extent of the SVAS stays in the same range, we can eventually look at going longer between exams.

I'm happy with the results, as was the cardiologist. We're praising the Lord for the grace He has shown Kieran so far. He is good -- all the time.

Ears and eyes

The audiologist gave Budy's hearing the all-clear (meaning he doesn't have the sensorineural hearing loss common to those with WS); however, the ophthamologist said he'll need to do something about Kieran's tear duct blockage sometime in the near future, namely stick a probe into the duct to clear it while K is sedated--an outpatient procedure. Fun times.

"At least it's not . . ."

There's a lot of sadness in hospitals, and as I watch the mother wheeling her baby attached to an oxygen machine or see a child with his bald head covered in a scarf, it's tempting for me to think, "Well, at least Buddy's not (fill in the blank)." Or "I'm thankful Buddy doesn't have (fill in the blank)." As if, if he did have (fill in the blank), I wouldn't be able to bear it.

However, I don't believe this is a beneficial -- or biblical -- response to the situations I find myself in . . . for several reasons.

1. It denies the sufficiency of God's grace. God's grace is sufficient for every situation we're in--if Buddy were (fill in the blank), we would have the grace and strength to deal with it. As it is, God sheds His mercy on us every day, enabling us to deal with whatever needs to be dealt with.

2. It diminishes the value of the person who has (fill in the blank). Imagine hearing someone say, "Well, I'm thankful, at least, that my child isn't like (insert your name here)." Each person is an image-bearer of the Creator, and although that image is now marred by the curse of sin, we still retain the fingerprint of God in our lives. All of His children are precious in His sight. And we all stand before Him equally in need of His loving salvation.

3. It's not a very comforting thing to say--or think--anyway. Imagine that you're a person with (fill in the blank). How do I comfort this person? "Well, at least you're not ___." And then that person? . . . What about the last person at the bottom of the heap? How do I comfort them?

4. Proverbs 14:10 is true. Each heart knows its own bitterness. While I can sympathize, and even empathize, with someone else's pain, I can't truly know what they're going through. And thinking or saying this only serves to show that I know nothing of God's grace.

5. My response has already been determined by God: "Be joyful always; pray continually; give thanks in all circumstances, for this is God's will for you in Christ Jesus" (1 Thessalonians 5:16-18). I don't believe this means "find something to be thankful for in all circumstances," but instead that I am, in fact, to give thanks for each circumstance because it is God's will for me. Although God isn't the cause of the trials I face (my sin in Adam began that string of happenings), He is sovereign over His creation and ordains what comes to pass. In the midst of my grief, I am to joyfully give thanks for what He has brought about: "Thank you, Lord, that Kieran has Williams Syndrome. May we use this to bring You glory and to manifest Your grace and mercy."

Do you agree? Disagree? Remain indifferent? I invite your thoughts.

Now that I'm done pontificating, I'll let you know that the doctor was pleased with the results of Buddy's echocardiogram and says we are to continue doing what we do. He wants to schedule another one in six months, but at this point, isn't overly concerned about the supravalvular aortic stenosis.

Cardiology appointment

Buddy's service coordinator came today so that we can get started with the therapy sessions. She gave us a copy of the review the therapists did last week, and we set some goals for Buddy to accomplish within the next six months (Husband mandated that he be able to play football and speak in complete sentences) and chose an occupational therapist who will meet with us for an hour each week to help us accomplish those goals. We're set to begin sometime within the next few weeks.

After the coordinator left, I made some Jello. I've been under general anesthesia twice (there's a connection between this and the Jello -- trust me). Once in high school when I had my wisdom teeth taken out. (Immediately after that, I had to read Crime and Punishment for an English assignment--talk about being in pain.)

The second time was after I had Buddy yanked from my abdomen. I'd obviously had an epidural for the C-section, but after he was taken out and they were stitching me up, for some reason, I felt the need to move my legs, and I began to squirm on the operation table. Obviously not the best thing to do when a doctor has a needle in her hand. So the anesthesiologist (who was wearing a Steelers bandana in Husband's honor) put me under for about 10 minutes. When I woke up as I was being wheeled back to our room, I uttered these prophetic words, "I think I fell asleep for a bit." Husband just patted my hand.

At any rate, in his 10 (soon to be 11) short months, Buddy has also been sedated twice. And tomorrow will make the third time. He has another echocardiogram on his heart at 1 pm (to check if the aortic stenosis is progressing) and isn't allowed to eat anything (including formula) for 6 hours before the sedation. He is, however, allowed to have "clear liquids," which includes Jello, until 2 hours before he goes under. Since he doesn't like Pedialyte or broth, I thought I'd give the Jello a try and see if that keeps him satisfied. We shall see if he prefers "blue" or apricot -- or neither.

Trip to the geneticist

Buddy celebrated his 10-month birthday with a trip to the geneticist today. The doctor wanted to go over WS with us and answer our questions. We didn't learn much new today--nothing, at least, that we hadn't already found out from our Internet searches--but we'll be visiting with the geneticist more often in the future as he monitors Buddy's development and works with us on finding research studies for Buddy to participate in.

FISH results

Today, the genetics doctor called to let us know that the FISH test results confirmed that Buddy has Williams Syndrome and that his calcium levels are in the normal range (meaning he doesn't have the hypercalcemia usually associated with WS).

I had read in various places that many WS parents use melatonin to help their child sleep better at night. With WS, it's not the normal child-wakes-up-once-in-awhile-at-night thing. It's that child can't settle down at night and continues to wake every few hours. For example, tonight, I put him down to bed at 8:30. At 10:30, he's still not fully asleep, although he's quieted down and isn't crying anymore. And he'll probably be awake and crying again sometime between 1 and 4 (if not several times). It's not that he can't fall asleep on his own -- he does fine during his naps. For some reason--which I haven't been able to pinpoint with all the reading I've done--WS kids just can't self-soothe very well and nighttime sleeping (or lack thereof) is a huge issue. It's difficult to explain the difference between WS sleep issues and a non-WS child who simply wakes during the night, but if you've been here during the evenings/night, you know what I'm talking about. And, quite frankly, the WS diagnosis was somewhat of a relief in this particular area because it means we haven't been doing anything wrong (at least in this area)--the issue isn't with how we've taught him to sleep (and believe me, I've tried everything to get him to sleep better at night--including seriously considering calling in a "sleep expert")--it's with him.

At any rate, I asked the doctor about giving Buddy melatonin to help him sleep better, and the doctor was fine with it. So tomorrow I go on the hunt for melatonin, hoping that it gives us all a better night's rest.